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Book. 



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Copyriglitl^^ 



COPYRIGHT DEPOSm 



THE 
CASE HISTORY SERIES 



CASE HISTORIES IN MEDICINE 



Richard C. Cabot, M.D. 

Third edition, revised and enlarged 



CASE HISTORIES IN PEDIATRICS 

BY 

John Lovett Morse, M.D. 
Second edition, revised and enlarged 



ONE HUNDRED SURGICAL PROBLEMS 

BY 

James G. Mumford, M.D. 
Second Printing 



CASE HISTORIES IN NEUROLOGY 



E. W. Taylor, M.D. 
Second Printing 



CASE HISTORIES IN OBSTETRICS 



Robert L. DeNormandie, M.D. 

Second Edition 



CASE HISTORIES IN DISEASES OF WOMEN 

BY 

Charles M. Green, M.D. 



NEUROSYPHILIS 

MODERN SYSTEMATIC DIAGNOSIS AND TREATMENT 
Presented in one hundred and thirty-seven Case Histories 

BY 

E. E. Southard, M.D., Sc.D. 

AND 

H. C. Solomon, M.D. 

Being Monograph Number Two of the Psychopathic Hospital, Boston, 
Massachusetts. (Monograph Number One was A Point Scale for Measur- 
ing Mental Ability by Robert M. Yerkes, James W. Bridges and Rose 
S. Hardwick. Published by Warwick and York. Baltimore 1915.) 








Metchnikoff 
schaudinn 



Ehrlich 



Wassermann 

NOGUCHI 



NEUROSYPHILIS 

MODERN SYSTEMATIC DIAGNOSIS AND 
TREATMENT 

PRESENTED IN ONE HUNDRED AND THIRTY- 
SEVEN CASE HISTORIES 



E; E. SOUTHARD. M.D., ScD, 

BuIIaid Professor of Neuropathology, Harvard Medical School; Pathologist, Massachusetts 

Commission on Mental Diseases; Director, Psychopathic Department, 

Boston State Hospital ; Vice-President, American 

Medico-Psychological Association 



H. C. SOLOMON. M.D., 

Instructor in Neuropathology and in Psychiatry, Harvard Medical School ; Special Investi- 
gator in Brain Syphilis, Massachusetts Commission on Mental Diseases; Acting 
Chief-of-StaS, Psychopathic Department, Boston State Hospital 



WITH AN INTRODUCTION BY 
JAMES JACKSON PUTNAM, M.D.. 

Profettor Emeritut of Diseases of the Nervous System, Harvard Medical School 



BY VOTE OF THE TRUSTEES OF THE BOSTON STATE HOSPITAL 

MONOGRAPH NUMBER TWO 

OF THE 

PSYCHOPATHIC HOSPITAL, BOSTON, MASSACHUSETTS 



BOSTON 

W. M. LEONARD, Publisher 
1917 



0^ 



^(^%^^ 



Copyright, 1917, 
By W. M. Leonard 



JOi. 26 l^ib 

g)Cl.A499858 



MASSACHUSETTS 

A STATE THAT 

BOTH TOLERATES AND FOSTERS 

RESEARCH 



PREFACE 

This book is written primarily for the general practi- 
tioner and secondarily for the syphilographer, the neurologist, 
and the psychiatrist. Our material is drawn chiefly from a 
psychopathic hospital, that modern type of institution in 
which the mental problems of general medical practice come 
to a diagnostic head weeks, months, or years before the 
asylum is thought of. 

It is this peculiar nature of psychopathic hospital material 
— a concentrated essence of the most difficult daily problems 
of general practice — that brings together such an apparent 
mSlange of cases as are here described, ranging from mild 
single-symptom diseases like extraocular palsy up to genuine 
magazines of symptoms as in general paresis; from feeble- 
mindedness, apparently simple, up to apparently simple 
dotage, both feeble-mindedness and dotage really syphi- 
litic; from the mind-clear tabetic to the maniacal or deluded 
subject who looks physically perfectly fit; from the early 
secondaries to the late tertlaries or so-called quaternaries; 
from peracute to the most chronic of known conditions; 
from the most delicate character changes to the profoundest 
ruin of the psyche. 

Although the bulk of our case-material Is drawn from gen- 
eral practice through the thinnest of Intermediary membranes, 
the psychopathic hospital, yet we have tried to depict the 
whole story by presenting enough autopsled cases from district 
state hospitals to show exactly what treatment has to face. 
Nor have we hesitated to insert cases in which treatment 
has failed. 

In addition to (a) the Psychopathic Hospital, Boston, 
group of Incipient, doubtful, obscure, or complicated cases 
(the early clinical group) and (b) the Danvers State Hospital, 
Hathorne, group of longer-standing, committed, fatal cases 

5 



6 PREFACE 

(the finished or autopsied group) we present (c) a miscel- 
laneous group of cases, including many from private neuro- 
logical or psychiatric practice. No doubt those familiar with 
Boston medicine will see traces of the teaching of our former 
chiefs, notably Professors James Jackson Putnam and Ed- 
ward Wyllys Taylor. We are obliged to them for some well- 
observed cases. 

We have dedicated our work to the Commonwealth, but 
perhaps we should more specifically ascribe to the Massa- 
chusetts Commission on Mental Diseases (formerly the 
State Board of Insanity) the spirit that permitted our special 
study of neurosyphilis treatment. To these authorities, 
who have countenanced and encouraged a somewhat costly 
piece of special work since 191 4, we offer our thanks, hoping 
that other states will be one by one stimulated to the state- 
endowment of research. States doing full duty by research 
can be counted on one hand. 

To our Psychopathic Hospital colleagues and the Internes, 
and especially to Drs. Myrtelle M. Canavan and Douglas 
A. Thom of the Commission's Pathological Service, we also 
offer our best thanks. 

The Danvers traditions are tangible here: cases of Drs. A. 
M. Barrett, H. A. Cotton, H. W. Mitchell, H. M. Swift, and 
others are presented. We have been especially aided by the 
more recent work of Dr. Lawson G. Lowrey. 

Nor should we have been able to present our samples of 
brain correlation without drawing on the collection arranged 
and analyzed by Dr. Annie E. Taft, Custodian, Harvard De- 
partment of Neuropathology. The photographs, part of a 
collection of brain photographs now numbering over 10,000 
representing 700 brains of all sorts, were made by Mr. Herbert 
W. Taylor. 

The Wassermann testing work has been done by Dr. W. 
A. Hinton of the State Board of Health. Dr. HInton himself 
wrote out the text description of the Wassermann method. 
The method of his laboratory is held to the standards of con- 
trol set by previous chiefs, viz. by Professor F. P. Gay, who 
brought Immunological methods direct from the laboratory 
of Bordet (whose method the Wassermann method essentially 



PREFACE 7 

is), Prof. W. P. Lucas, and the late Dr. Emma W. D. Mooers, 
who had assisted Plaut in his first work with the Wassermann 
method in KraepeHn's Munich Clinic. 

The material combed by us to secure this illustrative series 
amounts to over 2000 cases of syphilis of the nervous system, 
including over 100 autopsies in all types of case. We have 
presented these with very varying fulness, chiefly to illustrate 
the contentions at the heads of the case-descriptions. 

In using the book, we suggest early reference to the Sum- 
mary and Key, where for convenience are placed numerous 
cross-references permitting extended illustration of almost 
every proposition from several cases. 

We have not made a large feature of the Medicolegal and 
Social section. This kind of thing well deserves a volume by 
itself, with all the legal and social-service implications drawn 
out in their amazing richness and detail. The social service 
slogan, "A paretic's child Is a syphllitic's child" has already 
accomplished a great deal of good in our local world. Some 
day we may not be compelled to drive the paretic's spouse and 
offspring to the Wassermann serum test! The general prac- 
titioner must help here. 

A note on the Treatment section. This is manifestly not 
the last word or even, we hope, our own last word, since the 
systematic work of the Massachusetts Commission must be 
kept up for some years to get a reliable verdict. Some of 
the results give rise to greater optimism than has prevailed 
in asylum circles, especially re general paresis. We are con- 
fident that no one can now successfully make a differential 
diagnosis between the paretic and the diffuse non-paretic forms 
of neurosyphilis in many phases of either disease, even with all 
laboratory refinements. If this be so, it is improper not to 
give the full benefits of modern treatment to all cases in which 
the diagnosis remains doubtful between the paretic and the 
diffuse non-paretic forms of neurosyphilis. We ourselves ad- 
vocate modern treatment, not only in the diffuse, but also 
in early paretic forms of neurosyphilis. 

It would have been out of place in a book in this Case 
History Series to have dealt extensively with the history of 
our topic. We have compensated inadequately for this lack 



8 PREFACE 

by a few remarks at the head of the Summary and Key. 
We are, like all others in the field, under the inevitable obli- 
gation to Nonne of Hamburg, whose great work has gone into 
three editions, the second of which has appeared in English 
translation (Nonne's Syphilis of the Nervous System, C. R. 
Ball, translator). Mott's work, embodied in a large volume 
of the ^Power-Murphy System of Syphilis, has also been at- 
tentively consulted, as well as the various systematic works 
on neurology and psychiatry. The topic of Neurosyphilis is 
getting wide and appropriate attention in this country through 
special journals, both those dealing with nervous and mental 
diseases, and those dealing with syphilis. Syphilis is in a 
sense the making of psychiatry and will go far to pushing 
psychiatry into general practice. 

At the last moment we have been led to deviate from our 
plan of presenting only local cases familiar and accessible to 
us. In a section on Neurosyphilis and the War, we present 
excerpts and digests of English, French, and German cases 
of neurosyphilis that have appeared in association with the 
war. Our own country has not suffered greatly as yet either 
from the lighting up of neurosyphilis under martial stress or 
from the immediate or remote effects of syphilis obtained 
in the unholy congress of Mars and Venus. Space forbids a 
large collection of these martial cases, but, as will be seen, a 
fair sample of problems is presented. 

Speaking for the moment as the senior author of this book, 
I wish to say that, were it not for the energy, industry, and 
ingenuity of the junior author, Dr. H. C. Solomon, the book 
would not have been written. Nor, in all probability, would 
the systematic work of the Commonwealth on neurosyphilis 
and its treatment ever have been begun. I can also accord 
the highest praise to Mrs. Maida Herman Solomon for her 
social-service work in this new field. 

Perhaps, in closing, we owe an apology to John Milton for 
our borrowings from the two Paradises. Had he known 
much about syphilis, Milton might have written still stronger 
mottoes for us. 

E. E. Southard 

74 Fenwood Road 
Boston, Massachusetts 



TABLE OF CONTENTS 

Page 

Section I. The Nature and Forms of Syphilis of the Nervous Sys- 
tem (Neurosyphilis). Cases i to 8 17 

Case 

1. Paradigm: protean symptoms, nervous and mental. Autopsy, with 

meningeal, parenchymatous, and vascular lesions 17 

2. Tabes dorsalis (tabetic neurosyphilis). Autopsy 31 

3. General paresis (paretic neurosyphilis). Autopsy 37 

4. Cerebral thrombosis (vascular neurosyphilis). Autopsy 42 

5. Juvenile paresis (juvenile paretic neurosyphilis). Autopsy 45 

6. Extraocular palsy (focal meningeal neurosyphilis). Autopsy 50 

7. Gumma of brain (gummatous neurosyphilis). Autopsy 53 

8. Meningitis hypertrophica iecervicalis (gummatous neurosyphilis). 

Autopsy 56 

Section II. The Systematic Diagnosis of the Forms of Neurosyphilis. 

Cases 9 to 38 63 

Case 

9. Neurasthenia versus neurosyphilis 63 

10. Paretic neurosyphilis versus manic-depressive psychosis 68 

11. Neurosyphilis versus manic-depressive psychosis 71 

12. Dementia praecox versus neurosyphilis. Autopsy 74 

13. Neurosyphilis: negative Wassermann reaction (W. R.) of serum 77 

14. Diffuse neurosyphilis: six tests apt to run mild 80 

15. Paretic neurosyphilis: six tests strong 85 

16. Taboparesis (tabetic neurosyphilis): tests like those of paresis 92 

17. Paretic versus diffuse neurosyphilis: confusion re tests 97 

18. Vascular neurosyphilis: positive serum, negative fluid W. R loi 

19. Seizures in diffuse neurosyphilis 103 

20. Seizures in paretic neurosyphilis 106 

21. Aphasia in paretic neurosyphilis iii 

22. Aphasia in paretic neurosyphilis 115 

23. Remission in paretic neurosyphilis 117 

24. Remission in diffuse neurosyphilis 122 

25. Paresis sine paresi 126 

26. Paretic neurosyphilis. Autopsy 131 

27. Gummatous neurosyphilis. Operation 137 

28. Extraocular palsy (cranial neurosyphilis) 140 

29. Tabes dorsalis (tabetic neurosyphilis) : six tests apt to run mild 141 

30. Tabetic neurosyphilis, clinically atypical 143 

31. Cervical tabes 146 

32. Erb's syphilitic spastic paraplegia 147 

33. Syphilitic muscular atrophy 149 

9 



10 CONTENTS 

Case Page 

34. Neurosyphilis of the secondary period 151 

35. Juvenile paretic neurosyphilis: optic atrophy 154 

36. Juvenile paretic neurosyphilis I57 

37. Simple feeblemindedness, syphilitic I59 

38. Juvenile tabes 161 

Section III. Puzzles and Errors in the Diagnosis of Neurosyphilis 

(Including Non-syphilitic Cases). Cases 39-82 165 

Case 

39. Paretic versus diffuse neurosyphilis. Autopsy 165 

40. Paretic wersM^ vascular neurosyphilis, cerebellar. Autopsy 169 

41. Paretic z)er5M5 vascular neurosyphilis, cerebellar. Autopsy 172 

42. Tabetic combined with vascular neurosyphilis. Autopsy 175 

43. Tabetic neurosyphilis: mental symptoms, non-paretic. Autopsy.... 177 

44. Cerebral gliosis. Autopsy 180 

45. Neurasthenia versus neurosyphilis 183 

46. Hysteria. Neurosyphilis of the secondary period 185 

47. Manic-depressive psychosis versus paretic neurosyphilis 187 

48. Cerebral tumor 190 

49. Early post-infective paretic neurosyphilis 192 

50. Atypical paretic neurosyphilis, hemitremor. Autopsy 197 

51. Paretic neurosyphilis. Autopsy 199 

52. Manic-depressive psychosis versus paretic neurosyphilis 202 

53. Syphilitic (?) exophthalmic goitre. Autopsy 205 

54. Argyll- Robertson pupils 209 

55. Argyll- Robertson pupils: pineal tumor. Autopsy 212 

56. Neurosyphilis (?) with negative spinal fluid 216 

57. Disseminated syphilitic encephalitis, seven months post-infective. 

Autopsy 218 

58. " Pseudoparesis " 222 

59. Syphilitic paranoia? 225 

60. Paretic neurosyphilis versus alcoholic pseudoparesis 227 

61. Alcoholic pseudoparesis versus paretic neurosyphilis 231 

62. Alcoholic neuritis and paretic neurosyphilis 234 

63. Chronic alcoholism versus paretic neurosyphilis 236 

64. Neurosyphilis, diabetic pseudoparesis, or brain tumor 238 

65. Neurosyphilis and diabetes 240 

66. Neurosyphilis : hemianopsia 242 

67. Paretic neurosyphilis versus syphilis and cerebral malaria 245 

68. Paretic neurosyphilis: gold sol test "syphilitic." Autopsy 247 

69. Lues maligna 250 

70. Neurosyphilis versus multiple sclerosis 253 

71. Atypical neurosyphilis 256 

72. Huntington's chorea versus neurosyphilis 258 

73. Senile arteriosclerotic psychosis versus neurosyphilis 262 

74. Hysterical fugue versus neurosyphilis 264 

75. Tabetic neurosyphilis versus pernicious anemia 267 

76. Congenital neurosyphilis 270 

77. Congenital versus paretic neurosyphilis 272 

78. Juvenile paretic neurosyphilis 275 



CONTENTS 1 1 

Case Page 

79. Epilepsy versus juvenile neurosyphilis 277 

80. Addison's disease and juvenile paretic neurosyphilis. ,, Autopsy 279 

81. Neurosyphilis of the secondary period 283 

82. Taboparetic neurosyphilis and typhoid meningitis. Autopsy 284 

Section IV. Neurosyphilis, Medicolegal and Social. Cases 83-98 ... 289 
Case 

83. A public character, neurosyphilitic. Autopsy 289 

84. Debts, neurosyphilitic 295 

85. Suicidal attempt by a neurosyphilitic 296 

86. Neurosyphilis and juvenile delinquency 298 

87. Neurosyphilis in a defective delinquent 300 

88. Paresis sine paresi in a forger 303 

89. Trauma: juvenile paretic neurosyphilis 306 

90. Trauma: paretic neurosyphilis 308 

91. False claim for trauma: neurosyphilis 309 

92. Traumatic exacerbation? in neurosyphilis 310 

93. Trauma: cranial gumma at the site of injury 311 

94. Occupation-neurosis versus syphilitic neuritis 312 

95. Character change: neurosyphilis 314 

96. A neurosyphilitic family 316 

97. A neurosyphilitic's normal-looking family 318 

98. The neurosyphilitic's marriage 319 



Section V. The Treatment of Neurosyphilis. Cases 99-123. 

(Cases 99-103 show the Variety of Structural Lesions that 

Treatment has to Face) 323 

Case 

99. An incurable spastic paresis in paretic neurosyphilis. Autopsy 323 

100. A theoretically curable case. Autopsy 328 

loi. A highly meningitic case, theoretically amenable to treatment. 

Autopsy 332 

102. A highly atrophic case, theoretically not amenable to treatment. 

Autopsy 335 

103. Paretic neurosyphilis with markedly focal lesions. Autopsy 338 

(Cases 104 to 123 are Examples of Treatment Including Suc- 
cesses AND Failures.) 

104. Diffuse neurosyphilis: treatment successful after nine months 342 

105. Atypical neurosyphilis: treatment successful 346 

106. Argyll- Robertson pupil not necessarily of bad prognosis: treated case 

an insurance risk 350 

107. Spinal fluid cleared: symptoms persistent 355 

108. Arteriosclerosis does not contraindicate treatment 359 

109. Symptoms of intracranial pressure relieved by treatment 362 

no. Therapeutic improvement in tabetic neurosyphilis 366 

111. W. R. rendered negative in tabetic neurosyphilis 367 

112. Example of successful treatment of paretic neurosyphilis 37° 

113. Another example 37^ 



12 CONTENTS 

Case Page 

114. Clinical recovery but tests persistently positive in treated paretic 

neurosyphilis 375 

115. Improvement delayed in treated paretic neurosyphilis. 377 

116. Non-neural syphilis in treated paretic neurosyphilis. 380 

117. Partial recovery in treated paretic neurosyphilis 382 

118. Laboratory signs improved: clinical situation stationary: treated 

paretic neurosyphilis 384 

1 19. Another example 386 

120. Failure of treatment 388 

121. Treatment, at first mild, later intensive 390 

122. Intensive treatment 392 

123. Syphilitic feeblemindedness improved by treatment 395 

Section VI. Neurosyphilis and the War. 

Cases A to N from British, French, and German Writers 
(1914-1916) 
Case 

A. Tabes "shell-shocked" into paresis? (Donath) 401 

B. Latent syphilis "shell-shocked" into tabes? (Duco and Blum) .... 403 

C. Aggravation of neurosyphilis by service? (Weygandt) 404 

D. Aggravation of neurosyphilis by service? (Todd) 406 

E. Aggravation of neurosyphilis on service? (Todd) 409 

F. Duration of neurosyphilitic process important. (Farrar) 411 

G. Latent syphilis lighted up to paresis by war stress without shell- 

shock. (Marie) 412 

H. Paresis lighted up by "gassing"? (de Massary) 414 

I. Epilepsy in a neuropath lighted up by syphilis acquired at war. 

(Bonhoeffer) 415 

J. Syphilitic — after Dixmude epileptic. (Bonhoeffer) 417 

K. Syphilitic root-sciatica in a fire- works man. (Dejerine, Long) 418 

L. Paresis lighted up in civilian by domestic stress of the war. (Percy 

Smith) 420 

M. Shell-shock pseudoparesis. (Pitres and Marchand) 421 

N. Shell-shock pseudotabes. (Pitres and Marchand) 424 

Section VII. Summary and Key , 427 

Appendices: 

A. The six tests 471 

B. Common methods of treatment .... 486 



INTRODUCTION 

It is a privilege to be allowed to write a word of introduc- 
tion to a textbook which so richly fulfils its function as does 
this volume on the manifold disorders classified under Neuro- 
syphilis, a subject of which the importance for the welfare of 
society is found to loom the larger the more deeply its mys- 
teries are probed. 

The case-histories with which its pages are so amply 
stocked are carefully analyzed in accordance with a broadly 
chosen plan, and the generalizations that precede and follow 
them are obviously based on a wide and varied personal 
experience such as alone could render a familiarity with the 
literature of the subjects treated adequate to its best useful- 
ness. Both writers were indeed well adapted for this task. 
Dr. Southard, as everyone is aware, has long been a highly 
conscientious, ardent and productive worker in the depart- 
ment of pathological anatomy, and of late years a careful 
student of clinical diagnosis and methods, both at the Danvers 
State Hospital and still more, at the Psychopathic Hospital 
which he worked so hard to found; while Dr. Solomon's 
researches, in the special field of neurosyphilis, have been of 
the highest order. 

Undoubted as are the merits of the case-system of in- 
struction that has been so much in vogue in recent years, 
and excellent as is the modern supplementation of this method 
by the use of published records, the danger is still real that 
the student will have presented to him a picture of nature in 
disease that is too diagrammatic, too concise, with the result 
that while the task of memory is lightened through simplified 
formulation, the training of the doubting and inquiring 
instincts is often given too little stimulus and scope. In 
this book this danger is deliberately met through the cast- 
ing of emphasis rather on the pluralistic aspects of the pro- 
cesses at stake than (primarily) on their unitary aspects. 

13 



14 INTRODUCTION 

The student who utiHzes this volume cannot but emerge 
from his study a more thoughtful person than he was at the 
period of his entry. He will have seen that clinical rules of 
thumb cannot be followed to advantage, and that, on the 
contrary, surprises are to be expected and prepared for. 
Let the recognition of this fact, if it seems to increase the 
difficulties in the way of diagnosis, not lead to pessimism in 
that respect, or to hopelessness In therapeutics. On the 
contrary the writers' bias Is towards the worth-whileness of 
clinical efforts and an Increased respect for accuracy and 
thoroughness in the utilization of modern methods of research. 
The chance Is indeed held open that even the gaunt spectre 
of "General Paresis" may prove to be less terrible than it 
seems, and for this hope good grounds are given- 
It Is In this way made clear, on the strength of anatomical 
evidence of much Interest, that even if In the treatment of a 
given patient, the time arrives when a fatal or unfavorable 
result seems manifestly foreshadowed. It may be still worth 
while to renew the treatment with fresh zeal, for the sake of 
combatting some symptom or exacerbation, for which a 
locally fresh process furnishes the cause. 

Another noteworthy principle here emphasized and Illus- 
trated is that the relationship between "functional" (hys- 
terical, neurasthenic, migralnoid) symptoms and the signs 
(or symptoms) of organic processes Is clinically important 
and worthy of much further study. This is a matter which, 
in a general sense, has Interested me for many years. Above 
and over the "organic" hovers always the "functional," as 
representing the first Indication of the marvelous tendency 
to repair, or substitution, for which the resources of nature 
are so vast. Yet this functional tendency also has its laws, 
of which. In their turn, the organic processes display the 
action in quasi diagrammatic form. Hysteria, neurasthenia, 
migraine, etc., do not arise de novo in each case, but conform 
to typical, though not rigid, formulas, susceptible of descrip- 
tion. I have recently had the opportunity to study in detail 
an analogous series of transitions between the movements 
(and emotions) indicative of apparently purposeless myo- 
clonic movements (on an epileptoid basis) and the movements 



INTRODUCTION 1 5 

of surprise, engrossment, purposeful effort, the excitement 
and joy by which the former were excited and into which 
they shaded over. 

Taken altogether, this book represents work and thought 
in which, for amount and kind, the neurologists of Boston 
may take just pride. 

James J. Putnam. 

St. Hubert's, Keene Valley, New York. 
August, 1917. 



Me miserable! which way shall I fly 
Infinite wrath and infinite despair? 
Which way I fly is Hell; myself am Hell; 
And, in the lowest deep, a lower deep 
Still threatening to devour me opens wide, 
To which the Hell I suffer seems a Heaven. 



Paradise Lost, Book IV, lines 73-78. 



I. THE NATURE AND FORMS OF SYPHILIS OF 
THE NERVOUS SYSTEM (NEUROSYPHILIS) 



PARADIGM to show possible abundance and 
variety of symptoms and lesions in DIFFUSE 
NEUROSYPHILIS ^ (" cerebrospinal syphilis »)• 
Autopsy. 



Case I. Mrs. Alice Morton* was in the hands of at least 
five well-known specialists in different branches of medicine 
and surgery during the nineteen years of her disease. It 
appears that she acquired syphilis upon marriage at the age 
of 23 to a man who later became tabetic and acknowledged 
syphilitic infection previous to marriage. Mrs. Morton 
remained without children and there were no miscarriages. 

At the age of 27, she developed iritis, paresis of the left eye 
muscles, and ulceration of the throat, with destruction of the 
uvula. The syphilitic nature of her disease was at once 
recognized and the classical treatment was given, although, 
through numerous shifts in consultants, this treatment was 
never pushed to the limit. At 28 Mrs. M. began to suffer 
from severe headaches resembling migraine and accompanied 
by attacks of parsesthesia; at 35, came severe pains in the 
back and difficulty in walking. 

At 36, the migraine attacks began to be accompanied by 
blurring of vision and dizziness. The difficulty in walking 
became extreme, affecting particularly the right foot. The 

* The cases chosen to illustrate the propositions of the 
boxed headings always illustrate several other points. See 
the footnotes of Section VI for lists of cases illustrating 
special points. The names assigned to the cases are fictitious 
and chosen to suggest race or descent. 

17 



1 8 FORMS OF NEUROSYPHILIS 

legs became spastic, there were pains and hyperaesthesia of 
the chest, and severe cramps of the legs. Anti-syphilitic 
treatment at this time yielded marked improvement. 

During her thirty-sixth year, Mrs. M. sustained curious 
transient losses of vision and of hearing. She was also ir- 
ritable, and at this time developed her first pronounced mental 
symptoms, namely, delusions concerning her relatives. 
There were also a few seizures of an epileptiform nature. 
At 38 there was a spell of total deafness, followed by im- 
provement. The eye muscles were also subject to a varia- 
ble involvement with intervening spells of improvement. 
The knee-jerks were lost, but} after a time returned in less 
pronounced form. Shortly, an absolute paralysis and ex- 
tensive decubitus developed, and death occurred at 39. 

The autopsy is briefly summarized below, but it is important 
in the understanding of Mrs. M.'s case (particularly some of 
the sensory symptoms and the transiency of certain symptoms) 
to consider the pre-infective history. Although there seems 
to be no doubt that the patient acquired syphilis at about 
23 years of age from a syphilitic husband, who himself later 
became tabetic, yet it is of note that the patient was the 
only child of parents, both of whom also suffered from mental 
disease. Mrs. M.'s father died of what was called softening 
of the brain (one should avoid terming all old cases of so-called 
" softening of the brain " syphilitic, since the older diag- 
nosticians did not always distinguish between non-syphilitic 
arteriosclerotic effects and syphilitic disease). Mrs. M.'s 
mother also died insane (confusion and emotional depression). 
It is clear, then, that we do not need to suppose that every 
symptom shown by Mrs. M. is directly due to destructive or 
irritative lesions immediately due to the spirocheta pallida. 
The case is, in fact, an excellent lesson as to the association of 
structural and functional effects in neuropathological cases. 
Mrs. M. as a child had shown talent, but was somewhat 
nervous and eccentric. At one time, she had an attack of 
hysterical dysphasia; at another time, an attack of hysterical 
dyspnea; during another period, an apparent obsession (kick- 
ing the mopboard at regular intervals). Moreover, she had 
for years suffered from migraine of a severe and unusual type. 



FORMS OF NEUROSYPHILIS 1 9 

Both the hysterical tendency and the migrainous tendency 
became mingled with the results of the neurosyphilis in later 
stages of the disease in such wise that it was hard to tell 
exactly where the structural phenomena left off and the 
functional phenomena began. 

For example, at the age of 32, nine years after infection 
and four years after the earliest nerve symptoms traceable 
to syphilis, and at about the time of the onset of spinal cord 
symptoms, an attack was described as follows: 

The patient had a very severe attack of migraine (?) 
yesterday, preceded and accompanied by paraphasia, 
so severe that for three hours she was unable to make 
herself understood, and indeed felt "as if her ideas were 
getting away from her." This attack was ushered in 
by a numbness of the forefinger and thumb of the right 
hand, which lasted for about three hours, though the 
earlier attacks had lasted for only about ten minutes. 
During this period the hand felt as if it had been frozen 
and the loss of muscular power was so great that she 
was unable to hold objects in the hand. In some of 
the attacks this parsesthesia has affected the entire left 
half of the body, and occasionally the right half. Some- 
times the seizures come on with great suddenness, so that 
once, when she was attacked while in the middle of 
the street, she had considerable difficulty in reaching 
the sidewalk. After the worst part of the attack is 
over a certain amount of paraphasia may persist for 
some days, together with awkwardness in the use of 
the right hand and numbness. She has had a great 
deal of nausea and vomiting, without reference to the 
taking of food.* 

Bearing in mind the mingling of structural with functional 
symptoms in this case, let us consider the autopsy findings. 

Peripheral neurosyphilis: The lesions of the cranial 
nerves were characteristically asymmetrical. Whereas the 
left third nerve looked entirely normal, the right third nerve 
had its diameter reduced two-thirds. On the other hand, 
the fourth nerves were equal and apparently normal. The 
sensory portion of the left fifth nerve was normal ; the right 
fifth nerve was normal. The right sixth nerve agreed with the 

* Notes of Dr. James J. Putnam. 



20 FORMS OF NEUROSYPHILIS 



ANATOMICAL 

FORMS OF NEUROSYPHILIS 

AUTONOMIC (SYMPATHETIC) NEUROSYPHILIS? 

PERIPHERAL NEUROSYPHILIS 

CENTRAL NEUROSYPHILIS 
MENINGEAL 
VASCULAR 
PARENCHYMATOUS 
MENINGOVASCULAR 
VASCULOPARENCHYMATOUS 
DIFFUSE ( = MENINGOVASCULOPARENCHYMATOUS) 

GUMMA 



Chart i 



FORMS OF NEUROSYPHILIS 21 



CLINICAL FORMS OF NEUROSYPHILIS 

HEAD AND FEARNSIDES, 1914 

SYPHILIS MENINGOVASCULARIS 
CEREBRAL FORMS 
HEMIPLEGIA 

AFFECTION OF THE CRANIAL NERVES 
MUSCULAR ATROPHY 

LATERAL AND COMBINED DEGENERATIONS 
EPILEPSY 

SYPHILIS CENTRALIS, 

DEMENTIA PARALYTICA 
TABES DORSALIS 
MUSCULAR ATROPHY 
OPTIC ATROPHY 
GASTRIC CRISES 
EPILEPTIC MANIFESTATIONS 



Chart 2 



22 FORMS OF NEUROSYPHILIS 

right third nerve in being atrophic, and was in fact reduced 
to a mere thread without contained nerve fibres at a point 
2 mm. from its superficial origin. Although the right third 
nerve was atrophic, it was the left seventh and eighth 
nerves which had become atrophic; the process had spared 
the right seventh and eighth nerves. The remainder of the 
cranial nerves were grossly normal, except that the optic 
nerves had an outer zone of a translucent nature. So far, 
no spirochetes have been demonstrated in any portion of the 
nervous system of this case, but such asymmetrical and focal 
cranial nerve lesions are perhaps due to local spirochetal 
infection, punctuating (as it were) the diffuse process. 

How much of the transient blindness, deafness, and 
ocular paralysis can be explained on the anatomical findings 
in these nerves? Possibly a portion of the phenomena can be 
so explained. Thus, the mechanical conditions of pressure 
inside and outside these nerves, both in their peripheral course 
' and in their passage through the membranes, can be readily 
{understood to differ during the acute and subacute inflam- 
jmation, during the process of repair in the pial tissues, and 
during the process of overgrowth of neuroglia tissue about 
the superficial origins of the nerves. Of course, the majority 
of lesions of these nerves were entirely extinct at the time of 
the autopsy, and their history could be surmised only from 
the appearances in the left eighth nerve. Here occurred a 
sharply marked focal area of gliosis with apparently total 
destruction of nerve fibres and related with a lymphocytosis 
of the investing membrane (one of the few areas of lymphocy- 
tosis found anywhere in this case). 

If it were not for the pre-infective history, the hysterical 
dysphasia and dypsnea, the youthful obsessions, the migrain- 
ous tendency, and the psychopathic inheritance, we might be 
tempted to try to explain the transient blindness, the deafness, 
and ocular palsies on the basis of mechanical and toxic vari- 
ations in the conditions of the peripheral cranial nerves. 
The existence of a trace of lymphocytosis in the left eighth 
nerve leads to the hypothesis that treatment might still be 
effective in this particular region (see below in discussion of 
spinal symptoms). 



FORMS OF NEUROSYPHILIS 23 

Spinal neurosyphilis: Not only the spinal cord but also 
the posterior and anterior nerve roots exhibited severe lesions. 
These lesions were both meningeal and parenchymatous. 
The meningeal process differed in its intensity in different 
parts of the spinal cord, being severest in the thoracic region. 
At one point in this region, the dura mater was so firmly 
attached to the pia mater that the line of demarcation be- 
tween the two membranes was hard to make out. In fact, 
it seems clear that there could have been no free intercom- 
munication between the spinal fluid above these adhesions 
of dura to pia mater and the spinal fluid below the adhesions. 
Accordingly, it seems that lumbar puncture, had it been 
practised in this case, would have failed to show features rep- 
resentative of the whole cerebrospinal fluid system. More- 
over, since at no point in this region of adhesions or in the 
pia mater of the spinal cord below this point, were found any 
lymphocytes, it seems clear that the ordinary lumbar punc- 
ture would have failed to reveal a pleocytosis. Whether this 
fluid would have yielded a positive globulin and excess al- 
bumin test, it is now impossible to say; but it appears that 
the process in the lower part of the spinal cord was to all 
intents and purposes extinct. 

However, there was one region of more severe inflammatory 
involvement. The spinal cord in the cervical region showed 
a lymphocyte infiltration of its vessels amounting to a mild 
myelitis (meaning, thereby, an inflammatory process of the 
spinal cord remote from the pia mater). Moreover, in this 
region, there was, besides the perivascular infiltration of the 
substance, also an infiltration of the overlying membranes 
themselves, especially in and near the posterior root zones. 

The lessons of this finding are several: The inflammatory 
process in this case does not appear to have been entirely 
extinct! Can we not suppose that treatment might still 
have benefited this local inflammation (perivascular infiltration 
of the cervical spinal cord substance and overlying lym- 
phocytic meningitis)? Can we not also picture the gradual 
ascent of the inflammatory lesions from lower segments to 
higher segments and possibly conceive of the gradual eleva- 
tion of the zone of hyperesthesia manifested in this case as 



24 FORMS OF NEUROSYPHILIS 

following the gradual displacement upward of the lymphocytic 
process? Are there spirochetes in this tissue? So far none 
have been discovered, possibly through inaccuracies of 
available technique. To the neuropathologist, however, the 
lesion looks like a local reaction to organisms. 

In addition to the spinal meningitis, chronic and acute, 
as above described, there were extensive parenchymatous 
spinal lesions. 

In the first place, the meningitis had affected practically 
all the posterior roots so that the explanation of the posterior 
column sclerosis of this case is clear. The meningitis had 
apparently been so marked, also, that all the fibres anywhere 
near the periphery of the spinal cord had been likewise de- 
stroyed. The posterior columns and the posterior root zones 
were markedly sclerotic ; or as we say (having reference to the 
overgrowth of neuroglia tissue) gliotic. But there was as 
much sclerosis (gliosis) of the lateral columns (particularly in 
the posterior two-thirds) as there was in the posterior columns 
and root zones. In fact, the entire posterior half or two-thirds 
of the spinal cord markedly outstripped the anterior portions 
of the cord in the severity of the gliosis (sclerosis) shown. 
But although we can explain the posterior column sclerosis, 
the sclerosis of the posterior root zones and the marginal 
sclerosis {Randsklerose) round the entire periphery of the 
cord, on the basis of long-standing effects of old meningitis, 
we cannot thus explain another finding, namely, the destruc- 
tion of the fibres in the lateral columns. This, in fact, 
is explained through lesions (mentioned below) that affected 
the encephalon. The net result of all these lesions of the 
spinal cord was to leave only the gray matter and a small 
amount of surrounding fibres (belonging to short tracts uniting 
near-by segments) intact. Briefly stated, every long tract in 
the spinal cord appeared upon examination to be extensively 
degenerated. The genesis of this parenchymatous loss was, 
however, double, being in part due to a local meningeal proc- 
ess (sometimes known as " perimeningitis ") and in part due 
j to a cutting off of the pyramidal tract fibres on both sides by 
I lesions higher up in the nervous system. 

Can we offer any explanation of the partial return of knee- 





^y.^^ 




Case i. Spinal Cord (Three Levels) Showing: 

A. Marginal sclerosis — effect of old meningitis now extinct. 

B. Posterior column sclerosis — effect of meningitis about posterior roots also now extinct. 

C. Bilateral pyramidal tract sclerosis — effect of cerebral thrombotic lesions. 

Note distortion of tissues in B and C, partly artificial (tissues in places difHuent). 



FORMS OF NEUROSYPHILIS 



25 



ANATOMICAL FORMULAE 


MENINGOVASCULOPARENCHYMATOUS INVOLVEMENT 


M, V, P, or Combinations AppUed to the Classification 


of Head and Feamsides 




L SYPHILIS MENINGOVASCULARIS 




CEREBRAL FORMS 


MorVorMV* 


HEMIPLEGIA 


V 


AFFECTION OF THE CRANIAL NERVES M | 


MUSCULAR ATROPHY 


M 


LATERAL AND COMBINED DEGENE- 




RATIONS 


M 


EPILEPSY 


MorV 


II. SYPHILIS CENTRALIS 




DEMENTIA PARALYTICA 


MVPorVP 


TABES DORSALIS 


MP 


MUSCULAR ATROPHY 


P 


OPTIC ATROPHY 


P 


GASTRIC CRISES 


(M?or)P? 


EPILEPTIC MANIFESTATIONS 


P? 


* M = meningeal 




V = vascular 




P = parenchymatous 






Chart 3 



26 FORMS OF NEUROSYPHILIS 

jerks after their temporary total loss at a certain period of 
the disease? We may assume that the knee-jerks were 
functionally lost about a year before the death of the patient 
through the partial or even almost complete destruction of 
the entering posterior root fibres at that level of the spinal 
cord which is directly related with the knee-jerk. The later 
partial return of the knee-jerks apparently requires us to 
suppose the maintenance of some fibres and collaterals by 
which a functional connection can be effected between the 
fibres of the posterior roots and the anterior horn cells which 
innervate the quadriceps femoris. Let us now suppose 
that pari passu with the actual return of the knee-jerks, 
the destructive processes that are affecting both pyramidal 
tracts high up in the nervous system are now advancing. 
It is clear that, whatever inhibitory influence these pyramidal 
tracts have been exerting up to this time upon the knee-jerk 
reflex arc, that influence is now to be decidedly reduced in 
amount and possibly absolutely lost. Upon the loss of 
such inhibitory influences exerted from above, the few per- 
sisting connections of the posterior roots and anterior horn 
cells are now permitted to resume their functions. 

Encephalic neurosyphilis: The lesions mentioned above 
as causing destruction of the pyramidal tracts of the spinal 
cord were symmetrically destructive and atrophic lesions of 
the gray matter of both corpora striata with atrophy of the 
anterior segments of the internal capsules. There was a 
degenerative process of the corpus callosum especially af- 
fecting the forceps minor of the tapetum. The ventricles 
were largely dilated, indicating a considerable destruction and 
atrophy of the white matter in general. 

After the above discussion of the possible effects of py- 
ramidal tract lesion in this case, it is unnecessary further to 
discuss the paraplegia produced by the cystic lesions of the 
corpora striata. The theorist might inquire how these 
cystic lesions are produced : whether by vascular blocking or 
by toxic effects of the accumulations of spirochetes. Evi- 
dence is lacking which would completely sustain either hy- 
pothesis. Still, we do know that lesions almost identical in 
appearance may be produced by the necrosis consequent to 



FORMS OF NEUROSYPHILIS 27 

the plugging of nutritive vessels in an organ like the brain 
supplied with end arteries. Therefore, it is probable that 
most pathologists would believe these lesions of the corpora 
striata to be produced by vascular plugging of the nature of 
thrombosis. 

It is worth while to note that there was a suggestion of 
foci of encephalitis made out upon the gross examination. 
The cortex in general showed strikingly few lesions. How- 
ever, the convolutions did show in places numerous ill-defined 
areas of hyperemia and slight swelling. These areas were of 
irregular distribution and only a few mm. or cm. in diameter. 
No gross vascular lesions were demonstrable in connection 
with these focal areas. Microscopically, however, venous 
plugs of polymorphonuclear leucocytes were found, and the 
local hyperemias were found to be largely due to venous con- 
gestion. However, very few polymorphonuclear leucocytes 
were found outside the blood vessels. 

The white matter of numerous convolutions showed mi- 
croscopically certain pale spots suggestive of an early atrophic 
process. Very possibly these represent a general tendency in 
the cerebrum to the same process of parenchymatous loss which 
had proceeded to such a marked degree in the spinal cord. 

There was a single large so-called cyst of softening in the 
cerebellum (1.5 mm. across by 0.5-7.5 cm. in depth). 

How far can we explain the symptoms of this case on the 
basis of these encephalic lesions? We can offer no correlation 
with the cerebellar lesion ; and possibly this lack of correlation 
is to be expected on account of its failure to affect the vermis. 
As to the cystic lesions of the corpora striata, their effect in 
producing paraplegia at the close of life is obvious, and their 
possible relation to the partial return of knee-jerks has been 
discussed. Literally amazing was the comparative integrity 
of the cortical gray matter of this case when the spinal cord 
and the interior structures of the encephalon had been sub- 
jected to such severe and numerous lesions. The only 
mental symptoms noted in the case were sundry delusions 
directed against the patient's relatives and a certain optimism 
which led the patient to cling as if with an obsession to the 
belief that in the end she would get well. 



28 FORMS OF NEUROSYPHILIS 



VARIOUS FORMS OF NEUROSYPHILIS 
COLLECTED FROM SEVERAL SOURCES 

MENINGEAL NEUROSYPHILIS (M) 

GUMMA OF DURA MATER M 

GUMMATOUS MENINGITIS (Pial) M 

SYPHILITIC MENINGITIS (Pial) M 
SYPHILITIC CRANIAL NERVE PALSIES (Primarily Pial) M 

SYPHILITIC BULBAR PALSY M 

SYPHILITIC ROOT-NEURITIS M 

SYPHILITIC TRANSVERSE MYELITIS M 

SYPHILITIC NEURITIS (Some Cases by Extension) M 

SYPHILITIC EPILEPSY (Some Cases) M 

SYPHILITIC MUSCULAR ATROPHY (Some Cases) M 

VASCULAR NEUROSYPHILIS (V) 

SYPHILITIC ARTERIOSCLEROSIS V 

SYPHILITIC CEREBRAL THROMBOSIS V 

SYPHILITIC APOPLEXY V 

ANEURYSM V 

SYPHILITIC EPILEPSY V 

PARENCHYMATOUS NEUROSYPHILIS (P) 

GUMMA P 

CEREBROSPINAL SCLEROSIS P 

SYPHILITIC PARANOIA P? 

SYPHILITIC CHOREA P 

SYPHILITIC EPILEPSY P 

TABETIC PSYCHOSIS P? 

SYPHILITIC MUSCULAR ATROPHY P 

SYPHILITIC NEURITIS P 

Chart 4A 



FORMS OF NEUROSYPHILIS 29 



MENINGOVASCULAR NEUROSYPHILIS (MV) 


CEREBRAL SYPHILIS 


MV 


CEREBROSPINAL SYPHILIS 


MV 


SYPHILITIC EPILEPSY 


MV 


MENINGOPARENCHYMATOUS NEUROSYPHnJS 


(MP) 


CEREBRAL SYPHILIS 


MP 


CEREBROSPINAL SYPHILIS 


MP 


TABES DORSALIS 


MP 


ERB'S SYPHILITIC SPASTIC SPINAL PALSY 


MP 


VASCULOPARENCHYMATOUS NEUROSYPHILIS 


(VP) 


CEREBRAL SYPHILIS 


VP 


CEREBROSPINAL SYPHILIS 


VP 


PARETIC NEUROSYPHILIS (GENERAL PARESIS) 


VP 


LISSAUER'S GENERAL PARESIS 


VP 


MENINGOVASCULOPARENCHYMATOUS NEUROSYPHHJS (MVP) 


CEREBRAL SYPHILIS 


MVP 


CEREBROSPINAL SYPHILIS 


MVP 


PARETIC NEUROSYPHILIS 


MVP 


TABOPARESIS 


MVP 


DOUBTFUL (TOXIC?, IRRITATIVE ?) NEUROSYPHHJS (?) 


"PARESIS SINE PARESI" 




SYPHILITIC NEURASTHENIA 




TABETIC PSYCHOSIS 




SYPHILITIC PARANOIA 




SYPHILITIC POLYURIA, POLYDIPSIA 




SYPHILITIC NEURALGIA 




Chart 4b 



30 FORMS OF NEUROSYPHILIS 

Summary: We have here dealt at length with a long- 
standing Diffuse Neurosyphilis affecting to some extent 
the entire meninges and producing a destruction of posterior 
column fibres and numerous other fibres of the spinal cord 
(tabetiform portion of the neurosyphilis picture). We have 
also found central lesions of the corpora striata affecting the 
destruction of both pyramidal tracts (paraplegic portion of 
the neurosyphilis picture). We have found evidences of 
acute inflammation (lymphocytosis) in the cervical region of 
the spinal cord and in the left eighth nerve (progressive 
inflammatory neurosyphilis picture). In short, we have 
presented a case of diffuse (menlngovasculoparenchymatous) 
neurosyphilis characterized by an ascending character in 
a course of at least i6 years; we have indicated a number 
of possible clinical correlations, not only with the major por- 
tion of the clinical course (symptoms of myelitis and pyramidal 
tract destruction), but we have also mentioned, merely for 
their suggestive value, a number of finer correlations be- 
tween histological findings and certain clinical features (no- 
tably transient losses of vision and hearing, and a partial 
return of the lost knee-jerks). Bearing in mind the clinical 
and anatomical findings of this case, we shall be able to discuss 
the cases that follow in a briefer and more condensed fashion. 



FORMS OF NEUROSYPHILIS 3I 



TABETIC NEUROSYPHILIS ("tabes dorsalis," 
" locomotor ataxia ") complicated by vascular 
neurosyphilis (hemiplegia). Autopsy. 



Case 2. Francis Garfield had been a successful lumberman 
and had enjoyed good health until his forty-fifth year. 
Suddenly one day, while walking on the street, Garfield lost 
the use of his legs and for a time was quite unable to walk. 
However, he recovered locomotion and after a time there 
was nothing wrong with his leg movements except a slight 
ataxia. 

At the age of 52 Garfield had to give up work. It appears 
that he had been becoming cranky, sometimes, for example, 
shouting, whistling and slamming doors, apparently to annoy 
the family. His intellectual capacity seemed to be main- 
tained, although his memory was slightly impaired. 

At 67 years there was an ill-defined seizure, followed a few 
days later by another seizure with aphasia (wrong words 
used and lack of understanding of things said). 

For years Garfield had been totally deaf in the right ear 
(following explosion of a gun?). Now, however, the left 
ear also showed a sensory impairment. Slight slurring of 
speech had been noticed first in the sixty-sixth year. 

Physically there was a slightly enlarged heart with ac- 
centuated second aortic sound and irregular rhythm. Neuro- 
logically, Inability to stand or walk; marked ataxia In his 
leg movements; upper extremities quite well controlled; 
the pupils were small and unequal, the left being larger than 
the right; although the reactions were difficult to test, the 
pupils seemed to react slightly to direct light stimuli; the 
knee-jerks were absent; tests for sensibility so far as could 
be determined did not show any abnormalities; there was 
much complaint of sharp pains in the legs. 

There is no doubt that we are here dealing with a case of 
Tabes Dorsalis plus certain complications due to Vascular 
Lesions. The case went on to death from rupture of aortic 



32 FORMS OF NEUROSYPHILIS 



MAIN FORMS OF NEUROSYPHILIS 

(CLASSIFICATION OF THIS BOOK) 



DIFFUSE NEUROSYPHILIS 

(non-vascular forms of " cerebral," " spinal " and ** cerebrospinal 
syphilis ") 

VASCULAR NEUROSYPHILIS 

(" cerebral arteriosclerosis," " cerebral thrombosis ") 

PARETIC NEUROSYPHILIS 

(" general paresis ") 

TABETIC NEUROSYPHILIS 

("tabes dorsalis") 

GUMMATOUS NEUROSYPHILIS 
(" gumma of membranes, of brain ") 

JUVENILE NEUROSYPHILIS 
(paretic, tabetic, diffuse) 



Chart 5 



FORMS OF NEUROSYPHILIS 



33 





POSSIBLE INVOLVEMENT 




BRAIN AND CORD SYPHILIS 




[Mjembranes, [V]essels, [P]arenchyma 


[MVP] 


EARLY, LATENT ?, SYMBIOSIS ?, ATTENUATION ? . . . . 


MVP 


CEREBRAL, CEREBROSPINAL SYPHILIS, PAR- 




ESIS MVP 


[M]VP 


PARESIS; SYPHILITIC ARTERIOSCLEROSIS VP 


M[V]P 


? SYPHILOTOXIN FROM MENINGITIS MP 


MV[P] 


SYPHILITIC MENINGITIS; CEREBRAL OR 




CEREBROSPINAL SYPHILIS MV 


[MV]P 


SYPHILOTOXIC ATROPHY OR SCLEROSIS P 


M[VP] 


SYPHILITIC MENINGITIS M 


[M]V[P] 


SYPHILITIC ARTERIOSCLEROSIS V 




M, V or P in brackets [ ] means not involved. 




Chart 6 



34 FORMS OF NEUROSYPHILIS 



NEUROSYPHILIS 

SIX TESTS 



BLOOD WASSERMANN 

SPINAL FLUID WASSERMANN 
CYTOLOGY 
GLOBULIN 
ALBUMIN 
GOLD SOL 



Chart 7 



FORMS OF NEUROSYPHILIS 35 

aneurysm (also doubtless a syphilitic complication). The 
death occurred at 71, four years after admission to Danvers 
Hospital. 

This case has been especially worked up and published by 
Dr. A. M. Barrett on account of the fact that the vascular 
lesions of the brain had produced a condition of pure word- 
deafness. Reference is made to the Journal of Nervous and 
Mental Disease, Vol. 37, 1910, for a complete description of 
the brain findings and an analysis of the word-deafness, a 
summary of which is as follows : 

" Reaction to Words and Sounds. — Total deafness to 
words spoken, but gives attention to sounds; no ability 
to recognize meaning of sounds heard; no ability to 
repeat words heard. Spontaneous Speech. — Retained 
ability to speak spontaneously, with rare paraphasic 
utterances; occasional inability to speak readily the 
word desired, but later always giving the correct reaction ; 
calculation fair; spelling good except for occasional 
paraphasia; spelling good for words pronounced. Re- 
action to Things Seen. — Objects correctly recognized 
and named except for an occasional paraphasic reply; 
mistakes in pronunciation not recognized ; correct color 
recognition. Reaction to Things Felt. — Good for 
familiar objects; an occasional paraphasic reply. Re- 
action to Words Seen. — Reads printing and writing 
understandingly ; unimpaired reading except for an 
occasional paraphasic reply; meaning of familiar signs 
recognized; slight difficulty in readily understanding 
meaning of arithmetical signs. Writing. — Spontane- 
ous writing and drawing ability retained; ataxia 
(tabetic) in writing movements; no ability to write 
from dictation. Internal language. — No evidence of 
impairment." 

The brain post mortem showed severe atheromatous de- 
generation of the arteries at the base of the brain. Both 
middle cerebral arteries showed scattered atheromatous 
patches. The pia mater was transparent and delicate, except 
in the regions of both Sylvian fissures. There were residuals 
of old softening in both temporal lobes. In the fresh brain 
the regions of the right and left first temporal convolutions 
were sunken inward, and the pia intimately adherent to the 



36 FORMS OF NEUROSYPHILIS 

softened areas. ..The limits and more exact localizing of these 
softenings were worked out from serial sections. 

Barrett found in his serial sections that, although the trans- 
verse temporal convolutions of the left hemispheres were in- 
tact, these convolutions were undermined throughout their 
entire extent by degenerations in the fibres of the center of 
the first temporal convolution. Barrett, accordingly, re- 
garded his case as essentially a case of subcortical tissue 
destruction. He agrees with various authors that the pure 
word-deafness of his case is the result of an isolation of the 
receiving station in the transverse convolutions of the left 
hemisphere. The tissue destruction produced by the vas- 
cular lesion had cut off the transverse convolutions from 
the internal geniculate body. 

We are here, however, not considering the origin and rela- 
tions of pure word-deafness but present the case as one of 
tabes dorsalis of 20 years standing, terminated by two char- 
acteristic syphilitic complications, first, an extensive destruc- 
tion of brain tissue through cerebral thrombosis and secondly, 
fatal aortic aneurysm. 

Summary : We have here dealt briefly with a long-standing 
case of Neurosyphilis of the Tabetic type: A characteristic 
but not necessary complication of the case is the Late Cere- 
bral Vascular Involvement. The posterior column sclerosis 
is virtually the only spinal change. Spinal meningeal changes 
are absent (although It is to be assumed that chronic inflam- 
matory changes in the posterior roots were at one time present 
in some quantity and although the spinal fluid characteristic- 
ally shows lymphocytosis in tabetic neurosyphilis). 

Whether the spirochetes produce special toxic components 
able to cause tabes or whether special kinds of spirochete are 
the tabes-making kinds is hard to say. Special qualities of 
individual tissue may be involved. 

The cerebral lesions of a cystic nature are of vascular 
origin, like the differently localized encephalic lesions of Case 
I (Alice Morton) . Vascular syphilis is not a special property 
of the vessels of the nervous system. In fact this very case 
died of aortic aneurysm. 



FORMS OF NEUROSYPHILIS 37 



PARETIC NEUROSYPHILIS (" general paresis," 
"dementia paralytica," "softening of the brain"). 
Autopsy. 



Case 3. James Dixon, 44, was first seen at the Danvers 
Hospital, reciting verses in a dramatic and noisy way. He 
remained good-natured and jolly; nor was there any change 
in his euphoria until he had become physically weaker and 
more generally demented. In fact, Dixon appeared to be- 
come more and more expansive as he became physically 
weaker. He was in the habit of describing himself as 
"0. K., No. I, Superfine." 

Physically the patient was gray and bald on vertex, had 
a dusky complexion, was very thin (6 ft. in height, weight 
155 lbs.); the mucous membranes were pallid; the teeth 
rather poorly preserved; the heart was somewhat enlarged; 
the pulse irregular In rhythm, of poor volume and tension. 

Neurologically, the patient showed a characteristic Rom- 
berg sign and ataxia in walking a straight line. The tremulous 
tongue was protruded to the left, and there was a coarse 
tremor of the extended fingers. The knee-jerks were absent, 
and the Achilles jerks could not be obtained; the plantar 
reactions were slight; the arm reflexes were present. The 
pupils were stiff to light. There was a marked vocal tremor. 
The sensations could not be tested on account of the patient's 
mental state. 

It appears that Dixon had left school at about 16, at about 
22 had gone into the provision business, and later had be- 
come a hotel clerk. He had married at 28 ; there had been 
two miscarriages, at three months and six weeks respectively ; 
one child was stillborn; four children were living. 

The patient was not very alcoholic. The patient's wife 
thought the symptoms had been coming on since his forty- 
first year when irritability set in, but he was not discharged 
from work until about a year since. He was taken back again 
after his wife's pleas, and remained at work about three 



38 FORMS OF NEUROSYPHILIS 

months; but for ten months before admission to the hospital, 
Dixon had done practically nothing, had shown a marked 
memory failure and speech defect, at the same time claiming 
to be a person capable of doing and accomplishing everything. 
He had become careless of his personal appearance, collected 
a drawer-full of stumps of cigars, carried lumps of coal in his 
pocket, laughed causelessly, and spat on the carpet. 

We here deal with a case of unknown duration from the 
initial infection, but with symptoms lasting about three 
years and three months. Aside from the cause of death 
(empyema of left pleural cavity associated with acute hemor- 
rhagic splenitis, acute ileitis, and bronchial lymphnoditis) , 
the body showed a number of other lesions outside the nervous 
system. There was the usual sclerosis of the aorta, though 
perhaps less marked than usual. There was a curious acute 
arteritis with fusiform dilatation of the arteria profunda 
femoris, with an edema of the thigh muscles and blebs of the 
overlying skin. There were also multiple chronic caseating 
lesions of the liver, without evidence of fibrosis. The explana- 
tion of these liver lesions Is not yet clear. There was a cloudy 
swelling of the kidney. 

The calvarlum was dense and the dura mater thick and 
adherent. There was a chronic leptomeningitis, which, how- 
ever, was rather unusual in being most marked In the posterior 
cisterna and along the sulci of the cerebellar hemispheres. 
There was a general cerebral sclerosis, with a question of 
atrophy of the superior temporal gyri (suggesting the so-called 
Lissauer's paresis). There was a marked cerebellar sclerosis 
with a consequent sclerosis (grossly palpable) of the commis- 
sural fibres of the pons. There was a generalized slight spinal 
sclerosis. As a fair sample of the variety of head findings in 
paretic neurosyphilis, the details of the head examination are 
presented. 

Crown bald, with a slight fuzzy growth of short hairs. 
Scalp slightly adherent to calvarlum; latter of usual 
thickness but denser than normal. Dura adherent 
to calvarlum in region of vertex ; dura not remarkable. 
Sinuses normal. Arachnoid villi moderately developed. 
Pia mater a trifle thickened and rather evenly through- 



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A. Normal postcentral cortex. 
(Compare B.) 



B. Nerve-cell losses. Perivascular 
deposits of mononuclear cells, amongst 
which are numerous plasma cells. Note 
decrease in number of nerve cells. Note 
irregular disposition of nerve cells. 
From paretic neurosyphilis. 



FORMS OF NEUROSYPHILIS 39 

out the cerebral portion. Linear sulcal markings are 
remarkable for their absence. The wall of the cerebello- 
medullary cisterna is thick and opaque. The most 
prominent pial thickenings are over the cerebellum. 
These are linear or may show feathery out-growths and 
are seated over the sulci, particularly in the neighbor- 
hood of the fissure and about the great cerebellar 
notch. They correspond fairly well with the focal 
variation in consistence of under-lying tissues noted 
below. 

Brain weight, 1265 grams. Consistence somewhat 
increased throughout and somewhat evenly increased. 
The prefrontal region shows the maximal increase of 
consistence but the remainder of the frontal region and 
corresponding occipital region are much firmer than 
normal. The two superior temporal gyri appear to be 
firmer than adjacent gyri and are possibly slightly 
diminished in superficial diameter. The hippocampal 
gyri are fairly firm. The substance on section is a 
trifle more moist than normal. The gray and white 
matter cut quite evenly. Diminution in depth of gray 
matter, if existent, could not be demonstrated. The 
ventricles show a moderate sanding throughout, best 
marked in the fourth ventricle. The basal ganglia are 
not remarkable except for the development of numerous 
dilated perivascular spaces about the lenticulostriate 
vessels. The pons is atrophic, but more so on the 
right side. The pons, like the prefrontal cortex, shows 
on section a distinct increase of consistence immedi- 
ately beneath the pia mater. The Avhite bands of the 
pons on section are distinctly firmer than the interven- 
ing substance. The olives are of equal consistence. 
Weight of cerebellum, pons, and medulla, 155 grams. 
The cerebellum shows an obvious atrophic and gliotic 
process of a symmetrical character. The superior 
surface, including both vermis and hemispheres, shows 
a consistence above normal and general reduction of 
the depth measured from the white matter. The re- 
duction in depth gives rise to a visible depression as 
compared with tissue posterior to the postclival sulci. 
The lobus cacuminis, though slightly raised from the 
surrounding lobes, is equally firm, if not firmer. The 
superior and inferior surfaces show practically an equal 
increase of consistence. The dentate nuclei are not 
especially increased in consistence. The fiocculi are 
reduced in size about one-third. 



40 FORMS OF NEUROSYPHILIS 

There was slight universal increase in consistence of 
spinal cord) best marked in lumbar region. 

Microscopic findings are here presented merely in 
sufficient detail to establish the diagnosis. The left 
superior frontal gyrus shows extensive and somewhat 
irregular cellular and fibrillar gliosis of the plexiform 
layer, together with an increase of thickened vessels 
having lymphocytes and plasma cells in their sheaths. 

The perivascular infiltrations are most extensive in 
the lower layers of the cortex. The lamination is in 
places thoroughly obscured, except that representatives 
of the layer of large external pyramids are almost 
always demonstrable. 

The layer of medium-sized pyramids has undergone 
more numerical loss of elements than have the other 
layers. 

Gliosis of white matter. 

Specimens from the cerebellum show a destructive 
process of great severity, but a little irregular in extent, 
affecting chiefly the Purkinje cell belt. The Purkinje 
cells are often absent throughout one side of a given 
lamina, and there has ensued a dense accumulation of 
neuroglia cells along a former Purkinje cell belt, to- 
gether with a considerable gliosis of the molecular layer. 
Considerable gliosis of the white matter, both diffuse 
and perivascular In distribution. 

Perivascular plasma cell infiltrations as in cerebrum, 
but largely meningeal or In the white matter. 

Sections from the corpora striata demonstrate a mild 
and early granular ependymltis, considerable subepen- 
dymal gliosis of cellular type, considerable perivascular 
gliosis in the white portions of the tissue, and a moder- 
ate infiltration of perivascular sheaths with pigmented 
cells, lymphocytes, and plasma cells. There Is little 
evidence of alteration in the nerve cells. Some are 
unevenly pigmented. 



Summary: We here present a case with numerous and 
widespread neurosyphilitic lesions. However, the gross cere- 
bral vascular complications of Case i (Alice Morton) and of 
Case 2 (Francis Garfield) are notably absent in James Dixon. 
Rather atypical (there seems to be always something atypical 
in cases of neurosyphilis !) are the liver lesions and arteritis of 
the leg, atypical, that is to say, for Paretic Neurosyphilis. 



"^ 



% 



Apparent new formation of small blood vessel. Photographed by Dr. A. M. Barrett. 




I 



4 



i 



Rod cells (Stabchenzellen) in paretic neurosyphilis. Photographed by Dr. A. M. Barrett. 




Granular ependymitis — microscopic appearance of a marked example of "sanding" 
of ventricle. 



FORMS OF NEUROSYPHILIS 4I 

Highly typical of paretic neurosyphilis and almost constant 
therein is the aortic sclerosis. 

Characteristic and constant In paretic neurosyphilis Is the 
Plasmocjrtosis and Lyraphocytosis, Perivascular in distribu- 
tion about small cortical vessels. There is also a character- 
istic (though characteristically less prominent) Plasmocytosis 
and Lymphocjrtosis, Meningeal in distribution. The pleocy- 
tosls of the spinal fluid, almost constant though variable in 
amount in life, is an indicator of the meningeal picture and 
less directly of the parenchymatous picture. 

Granular Ependymitis ("sanding" of ventricle floors) Is 
characteristic and may be regarded as part of the paren- 
chymatous picture. This ependymitis is an Indicator how 
chemical changes could be readily produced at least In the 
ventricular fluids, since the limiting membranes of the nerve 
tissue are here subject to multiple breaks. The " sanding " 
is a neuroglia reaction to these multiple small breaks (Wei- 
gert's explanation). 

Parenchymatous losses have led to Atrophy and Sclerosis, 
of very varying extent in different parts of the encephalon. 
The atrophy is characteristic in paretic neurosyphilis, but by 
no means constant. Numerous cases have come to autopsy 
without clearly defined gross atrophy. Sclerosis Is also char- 
acteristic and even more frequent than atrophy, doubtless 
because sclerosis represents an earlier phase of a process 
eventuating In gross atrophy. 

A Tabetiform Picture characterizes the spinal cord, but 
in this case the tabetic clinical picture did not precede the 
paretic clinical picture. We are consequently to regard the 
tabetic spinal process as incidental and on all fours with 
the Cerebellar and Pontine Atrophy, 



42 FORMS OF NEUROSYPHILIS 



VASCULAR NEUROSYPHILIS (" syphiHtic cere- 
bral thrombosis"). Autopsy. 



Case 4. James Pierce was an almshouse transfer to the 
Danvers Hospital in his fiftieth year. He died three years 
later. The accompanying brain pictures demonstrate so 
extensive a lesion of the left hemisphere that it is of great 
interest to determine if possible the genesis and course of his 
disease. It appears that syphilis had been acquired some- 
where about the age of 38 or 40, so that the total duration of 
the process was between 13 and 15 years. In Pierce's forty- 
third or forty-fourth year, he had a shock while walking in 
the streets of his native city, whereupon he was subsequently 
transferred to the Danvers Hospital, whose data have been 
summed up as follows (we are obliged to Dr. Charles T. 
Ryder for these data) : 

Neurological examination: Neuromuscular condition: 
Barely able to walk or stand without assistance; 
hemiplegia of right side; swings foot out and drags toe 
out and around in attempting to walk. Right hand 
held by side, flexed at right angle; fingers contracted 
and thumb thrown across palm. Can lift arm from 
side; practically no movements of forearms or fingers; 
atrophy of deltoid, arm, forearm, and hand. Mus- 
cular movements of left upper extremities fairly well 
performed; good strength. 

Cranial nerves: Refuses to respond to any tests to 
determine hearing or vision, but evidently hears what is 
said to him, and in his movements gives no evidence of 
deafness. Right corner of mouth droops; tongue pro- 
trudes straight. 

Reflexes: Pupils dilated; margins irregular; left 
pupil larger; they vary in size but it is impossible to 
determine whether the variation is due to light or ac- 
commodation reflex. Reflexes of right side extremely 
exaggerated throughout; there is little ankle clonus; 
Babinski is not obtained, patient holding his toes in 
flexed position in resisting attempts to elicit reflexes. 



FORMS OF NEUROSYPHILIS 43 

Sensations: Reaction to pain stimuli on either side. 
Evidently some anesthesia on right side, but pressure 
is apparently very painful. There is considerable 
spasticity of limbs on right side on passive motion. 
Too demented to make accurate tests. 

The above examination was made on May 6, 1904. 
On May 20th the record states: 

There is almost complete sensory aphasia with word 
deafness; some paraphasic circumlocution. Many of 
his words are very well enunciated but have no meaning. 
Is apparently unable to recognize objects or their uses. 

Brother stated that he was always supposed not to be 
over bright. Physician's certificate states that he is 
epileptic, averaging two attacks per week. On the 
15th of May he had a general convulsion; was uncon- 
scious for half an hour, and dull and drowsy for two 
hours afterwards. On the 19th, he had a similar attack 
in the afternoon, the convulsion lasting a minute, and 
he was stuporous for an hour. 

On November 8 th he had a severe epileptic convul- 
sion. His body was curled up to the right. The 
convulsive seizure lasted for two minutes and was 
followed by complete unconsciousness for an hour, 
when the patient roused and appeared as usual in a few 
minutes. From that time to December 15th he had 
five epileptic convulsions; he was much more feeble, 
and unable to help himself as much as formerly. 

Nov. 7, 1905: Patient has had occasional convul- 
sions since last note, but none during the last three 
months. He is confined to bed, has become very much 
demented, and shows very marked speech defect, so 
that he is almost unintelligible. He understands only 
the simplest directions. Legs are considerably con- 
tracted and knees are flexed. Arm and hand on the right 
are paralyzed and show some atrophic changes; par- 
tially flexed. Left elbow-jerk is very lively. On 
May 23, 1906 he was reported as having Achilles on 
right side only, and Babinski on right side. He died 
January 5, 1907. 

The autopsy findings were as follows: 

Head: Calvarium of moderate thickness; diploe 
present; dura slightly adherent over bregmatic region. 
Longitudinal sinus contains cruor clot. Dura is some- 
what thickened and slightly more opaque than normal. 
Pacchionian granulations, small but fairly numerous. 
Pia contains throughout a considerable excess of clear 



44 FORMS OF NEUROSYPHILIS 

serous fluid. The convolutions in general are of good 
breadth and proportion. There is an atrophic area 
roughly circular in outline and about 2 cm. in diameter 
in the posterior part of the right third frontal con- 
volution corresponding to Broca's area on the opposite 
hemisphere. The space thus formed is filled with edema 
held by the pia. On the left side is a similar subpial 
collection which covers the site of the posterior portions 
of all of the third frontal convolutions, parts of the 
lower end of the precentral convolution, and the whole 
of the first temporal convolution, which have disap- 
peared entirely. The basal vessels show slight changes. 

Cerebellum and basal ganglia are grossly normal. 

The spinal membranes are negative. The regions of 
the pyramidal tracts in the cord are firm, project 
slightly from surface of section, and are china white. 

Summary: Here is a picture made up almost purely of 
Vascular Neurosyphilis, with Secondary Spinal (Py- 
ramidal Tract) Changes. Doubtless the genesis of this 
picture is allied to that of Case I (Alice Morton) and to that 
of the terminal vascular complications in a tabetic, Case 2 
(Francis Garfield). 

The absence of meningeal and parenchymatous (i.e., out- 
side the region of necrosis produced by the vascular disease) 
lesions is characteristic of an important group of neuro- 
syphilitic diseases. It is clear that the case, although one of 
extensive lesions, is not one of diffuse lesions in the sense of 
Case I (Alice Morton). 

The spinal fluid picture in life may nevertheless show (as 
other cases amply demonstrate) a certain amount of lympho- 
cytosis and possibly plasmocytosis, together with a variety 
of other changes. Treatment might be expected to keep 
down these associated changes, although obviously the effects 
of the necrosis are final and definite. Franz in Washington 
has succeeded in "reeducating" some of these hemiplegics, 
employing lower mechanisms of the nervous system. 






Case 4. (See previous figure for brain lesion.) Three levels of the spinal cord showing 
unilateral pyramidal tract sclerosis, 10 years after cerebral thrombosiso 



FOEMS OF NEUROSYPHILIS 45 



JUVENILE PARETIC NEUROSYPHILIS (" juve- 
nile paresis "). Autopsy. 



Case 5. John Lawrence was an under-sized negro, who 
came under hospital observation when he was 23 years of 
age. There was some evidence that the patient's father was 
a neurosyphilitic although accurate data were out of the 
question. At all events, John had Hutchinsonian teeth, a for- 
ward bowing of the tibiae, and Argyll-Robertson pupils. 
These findings together with a history of backwardness at 
school seem to stamp the diagnosis. It seems that there had 
been a change for the worse from the age of 18, though the 
boy had been able to sell newspapers and black shoes up to 
within a year of his arrival at the hospital. During the last 
months of his life, he showed a general incoordination, with 
false movements suggesting those of a drunken person. There 
were numerous tremors, the glance was shifting, and there 
was a tendency to nystagmus. Some of these phenomena 
(taking into account that the Hutchinsonian teeth were not 
entirely typical and there was even at times some doubt as 
to whether the pupils were actually stiff) led to a question of 
the diagnosis multiple sclerosis. 

There was, however, little doubt that the case was one of 
juvenile paresis. Among the symptoms found at various 
times in this case are the following: disorientation for time, 
place and persons, confusion, with coarsely irrelevant replies to 
questions, ill-defined and transitory delusions of persecution, 
auditory, tactile, and visual hallucinations, and defective 
memory. 

Early in life, the patient had had a habit of falling asleep 
in school hours, and had experienced a number of falls at 
various times. During an attack of measles he had had a 
number of spasms, each of which lasted ten minutes or more. 

The autopsy showed death to be due to an early bronchial 
pneumonia. The thymus was persistent, measuring 3X2X 
.5 cm. The marrow of the femur was red. 



46 FORMS OF NEUROSYPHILIS 

There was a moderate degree of sclerosis of the aorta con- 
fined to a few plaques in the arch (not a characteristic syphil- 
itic scarring of the aorta). The spleen was small and had a 
thickened capsule. 

The majority of the lesions, however, were in the nervous 
system, and the following description is taken from the routine 
hospital records to exemplify the findings in a fairly charac- 
teristic case of Juvenile Paresis. 

Head: Scalp closely adherent to calvarium. Cal- 
varium heavy without diploe. Dura adherent to cal- 
varium in bregmatic region. Sinuses contain liquid 
blood. Arachnoidal villi in considerable quantity. Pia 
mater contains considerable clear fluid and shows diffuse 
haziness and focal thickenings. The diffuse haziness 
is almost universal and is best marked over the superior 
surface of the cerebellum. The focal thickenings are 
of general distribution over the veins of the sulci on the 
superior surface of the brim and are heaped up to form 
considerable linear mounds near the region of the arach- 
noidal villi. The superior surface of the cerebellum is 
traversed by similar linear mounds of fibrous tissue 
running at an angle to the laminae. There is no notable 
increase of fibrous tissue at the base. 

Brain : Weight 965 grams. The sulcation is roughly 
symmetrical except in the occipital poles where there is 
unusually rich and complex but shallow sulcation. The 
cortical substance is everywhere firmer than normal, 
but the sulci fail to flare notably. In a few places there 
is a focal increase of consistence of still greater degree 
with apparent local hypertrophy (or gliosis with in- 
crease of substance). These foci are in the right second 
temporal gyrus (3 cm. in diameter) and in the left first 
temporal gyrus (of same size but somewhat less firm) 
and are of a whitish, waxen appearance, being visible 
several feet away by reason of their color and apparent 
encroachment upon the adjacent sulci. The foci are 
sharply limited by the sulci laterally, but pale out 
gradually before and behind. 

The convolutions of the vertex show another type of 
lesion. The tissue of the greater part of the vertex 
resembles that of the flanks and base in being firmer 
than normal and of a grayish pink color. Behind the 
fissure of Rolando on the right side and behind the an- 
terior limits of the ascending frontal region on the left 



FORMS OF NEUROSYPHILIS 47 

side the brain tissue of the vertex becomes suddenly 
still firmer and of a yellowish gray color. This lesion 
disappears gradually into the occipital microgyria be- 
hind and the gyri gradually lose their yellowish tint. 
The lesion fades away gradually so that it fails to in- 
volve the temporal convolutions. 

The cerebral tissue cuts firmly and smoothly. The 
tissue of the frontal region is a little edematous. The 
white matter is of a normal appearance. The ependyma 
of all the ventricles is somewhat sanded. The fourth 
ventricle is most affected. 

The cerebellum is not edematous and is as firm as 
the normal olivary bodies. The cerebellar hemispheres 
are symmetrical and of a normal appearance, save that 
the laminae are slightly narrower than usual and very 
compactly set. The color, where not obscured by the 
haziness of the pia mater, is of a grayish pink somewhat 
suggestive of freshly tanned shoe leather. The sub- 
stance cuts smoothly and firmly. The dentate nuclei are 
unusually firm. The pons is small, but of the usual 
color. Lower structures normal except the cord which 
is small and shows curious deviations from the normal 
markings. The posterior horns and gray commissure 
are at many levels the only structures to preserve the 
normal gray appearance, so that the H or butterfly 
appearance is replaced by a crescent. At these levels 
traces of gray matter often stand out in the loci of the 
anterior horns. 



The important anatomical diagnoses in the nervous system 
are as follows: 

Atrophy of cerebrum, 965 grams (there is of course a 
question whether we are not dealing with a degree of cerebral 
hypoplasia) . 

Focal scleroses of cerebrum, suggesting the tuberous 
scleroses of Bourneville. 

Occipital microgyria. 

Cerebral and cerebellar gliosis. 

Chronic ependymitis. 

Gliosis of the gray matter of the spinal cord. 

Chronic diffuse and focal leptomeningitis. 

The microscopic examination confirmed the diagnosis of 
paresis. The hypertrophic nodules were of special interest. 



48 FORMS OF NEUROSYPHILIS 

They were found to be overlain by a characteristic though 
thin exudate of lymphocytes and plasma cells, together with 
pigmented cells. The nodules appeared to be supplied with 
an unusual number of vessels of small calibre, about which 
were a few lymphocytes. The large vessels and those with 
well-developed adventitise were surrounded by more numerous 
lymphocytes and by more focal accumulations of pigmented 
cells. The cortex in the middle of a nodule had almost lost 
its characteristic cortical layering. The cortex was here 
reduced (specimen from temporal lobe) to about one-quarter 
of its normal thickness, and was found to be composed largely 
of expanded neuroglia cells and vascular tissue, with a few 
nerve elements, small, shrunken, and dark-staining. The 
destructive process appeared to have borne hardest on the 
layer of internal large pyramids and the fusiform layer. 
There was, however, nowhere any evidence of focal necrosis 
such as ought to characterize a true gumma. The sections 
stained by the Marchi method failed to show evidence of 
fatty degeneration within the focus, although there was a 
marked diffuse accumulation of fatty granulations along the 
nerve fibres in the underlying white matter. A special study 
of the cerebellar material was made by one of the authors.* 
Occasional Purklnje cells showed the characteristic bi- 
nucleate condition, which has frequently been noted in 
recent literature. 

The cerebellum of this case was perhaps the most markedly 
diseased of all portions of the nervous system. As noted, the 
cerebellar tissue was exceedingly firm. How far the notable 
incoordination of the case (he was observed on staff rounds 
characteristically curled up In a heap, showing quite an unusual 
degree of general incoordination) was due to the cerebellar 
lesions, it is perhaps not possible to say. 

Summary: John Lawrence, Juvenile Paretic Neuro- 
syphilis, is a foil to Case 3 (James Dixon), paretic neuro- 
syphilis due to acquired syphilis. 

Both showed Cerebral Atrophy, but Lawrence the more 

* E. E. Southard : Lesions of the granule layer of the 
human cerebellum; Journal of Medical Research, XVI, 1907. 



FORMS OF NEUROSYPHILIS 49 

markedly because of hypoplasia incidental to the congenital 
origin of his condition. 

Whereas Dixon gave little or no sign of stigmata, Lawrence 
(besides being undersized, having suspicious teeth, and 
showing at autopsy a persistent thymus) showed a Hydro- 
myelia and curious trefoil shape to the spinal cord. Dixon 
on the other hand had liver lesions and arterial lesions of the 
leg. 

The suggestion of Tuberous Sclerosis in Lawrence is not 
found in Dixon ; but we have not found it elsewhere. Bour- 
neville did not describe tuberous sclerosis as syphilitic. 

Binucleate Purkinje cells emphasize the congenital source 
of the lesions in Lawrence. 

Plasmocjrtosis and Lymphoc3rtosis, Perivascular, and (less 
marked) Meningeal, are found in both the congenital and 
the acquired cases, as also parenchymatous changes, both 
nerve cell losses and gliosis. Both also show granular 
ependymitis. 

It is clear that, over and above the factors of destruction 
evident in both Lawrence and Dixon, the congenital case, 
Lawrence exhibits also the effects of arrest (in brief not 
merely atrophy but also hypoplasia). Early treatment is, 
therefore, theoretically indicated In the juvenile group, which 
means early diagnosis. Early diagnosis and treatment are 
still more to be recommended because these juvenile cases 
progress often very slowly at first. 



50 FORMS OF NEUROSYPHILIS 



FOCAL BASILAR MENINGEAL NEUROSYPH- 
ILIS (" syphilitic extraocular palsy," plus other 
symptoms). Autopsy. 



Case 6. Flora Black, a housewife of 43 years, had been 
tired out for a year but had been apparently in fair health. 
She awoke one day with double vision due to a left internal 
strabismus. The visual difficulty gradually passed away so 
that five months after the sudden seizure she was apparently 
quite well again. There was one exception: about three or 
four months after the attack of diplopia, Mrs. Black had begun 
to feel a kind of weakness in various parts of the face and there 
were also fairly definite paresthesise. In the sixth month 
after the initial attack, the patient began to be unable to 
chew and was fain to support the lower jaw with a bandage 
to aid in mastication. Deglutition was, however, quite un- 
affected and] there was never any regurgitation of food. 
There were pains in the face, the forehead and the back of 
the neck. 

Upon physical examination at entrance to a general hos- 
pital, no changes in the body at large were discoverable. 
There was a slight edema of the ankles, otherwise no sign 
of bodily disease. 

Conditions in the head were as follows: The facial lines 
were (notes by courtesy of Dr. E. W. Taylor) smoothed out ; 
both upper and lower eyelids and the corners of the mouth 
drooped slightly and more markedly on the left side. There 
was slight photophobia and considerable lachrymation. 
The patient was unable to pucker forehead, nose or mouth. 
The unsupported lower jaw fell and the patient was unable 
to open the mouth widely. The movements of the tongue 
were normally performed. Speech was mumbling. Sen- 
sations of touch, heat and cold were preserved all over the 
face except that the left cheek below the level of the mouth 
yielded a less accurate registration of tactile sensations. 
A hot test tube did not feel as hot in the lower left cheek' as 



FORMS OF NEUROSYPHILIS 5I 

elsewhere. Quinine and sugar could not be tasted over the 
left half of the tongue in front. Smell and hearing were also 
diminished on the left side. It appeared that there was a 
complete paralysis of the 5th and 7th nerves and a partial 
paralysis of the 8th, nth and 12th, as well as a defect in 
smell. 

The patient died suddenly, three weeks after admission, 
running a slight temperature during her stay. The autopsy 
showed (rather surprisingly) a double ovarian carcinoma 
with metastases into the retroperitoneal glands. Both 
kidneys were found to be riddled with nodules of carcinoma. 
The pelvic veins were thrombosed and there was a complete 
occlusion of the pulmonary artery. There was a riding em- 
bolus in the foramen ovale and there was coronary embolism. 

The striking nature of these complications and the interest 
of the case neurologically would warrant its publication in 
complete detail. We here present the case with utmost 
brevity as an example of a Syphilitic Cranial Neuritis 
by extension from the meninges. 

The brain was in general without change but there was 
a considerable exudate over the entire pontine region which 
had involved several cranial nerves. The 5th nerves, es- 
pecially the left, showed gross effects of the' inflammatory 
lesion. There seems to be little or no doubt that this neuritis 
was of syphilitic origin despite the complication of the case 
with carcinoma of the ovary and despite the fact that the 
case was observed and came to autopsy before the modern 
methods of systematic diagnosis could be applied. It is the 
best case available to us for the demonstration of a focal 
cranial nerve lesion of the type characteristic of neurosyphilis. 
We may well suppose that similar conditions would have been 
found at various stages in the development of Case l (Alice 
Morton). The pontine region of Case i was entirely free 
from lymphocytic exudate at the time of the autopsy. Pos- 
sibly the clearing up of the pontine pia mater In Case I was 
a therapeutic effect of the thorough treatment therein used. 
Whether a case like Mrs. Black's could be cured (aside from 
the ovarian carcinoma and its complications) by the Insti- 
tution of vigorous systematic treatment is a matter of doubt. 



52 FORMS OF NEUROSYPHILIS 

Still, in a general way, these cases of focal syphilitic neuritis 
are among the most favorable cases for treatment. 

Summary: We present the case of Flora Black to em- 
phasize how slight in extent and theoretically curable neuro- 
syphilis may be. We fear that Case i (Alice Morton) may 
present too unrelieved and pessimistic a picture. The 
extensive vascular lesions and complications of Alice Morton, 
of Case 2 (Francis Garfield), of Case 4 (James Pierce) arrest 
attention by the incurability of their residual effects (if we 
omit modern attempts at reeducation of lower arcs). On the 
other hand the unrelenting progress to destruction of impor- 
tant parenchymatous structures, as shown in the paretic 
James Dixon (Case 3) and his juvenile replica John Lawrence 
(Case 5), as well as in Alice Morton (Case i) and the tabetic 
Francis Garfield (Case 2), lead to a certain justifiable pessi- 
mism. For it is only the meningeal and fine vascular in- 
filtrations of these cases that we can theoretically hope to 
combat, probably by destroying the spirochetes in these 
meningeal and perivascular loci. We seem theoretically less 
able to stop the progress of the often highly systemic and 
symmetrical, parenchymatous lesions of the tabetic and 
paretic group. 

The condition in Flora Black is clearly much more hopeful, 
both being more focal and being almost purely meningeal 
and therefore accessible to therapy. 

The two cases which conclude our general survey of neuro- 
syphilis are also focal cases, one of gumma (Lecompte) and 
one of focal dural lesion (Wyman). 





I. Pons, normal except for focal infil- 
tration of left fifth nerve. 



2. Higher power view of infiltrated left 
fifth nerve. 







i«Mf#^^ 



.^->^*^ 






3. Detail of infiltrated left fifth nerve, 
showing: i, diffuse infiltration with mononu- 
clear cells; 2, perivascular infiltration; 3, 
strands of relatively unaffected nerve fibers. 



Microscopic appearances in Case 6. Extraocular palsy (focal meningeal syphilis, especially 
of left fifth nerve). Illustrates exquisite focality of the syphilitic process sometimes found, 
as well as its unilaterality (giving rise to asymmetrical symptoms and signs). Process in 
itself probably curable. 



FORMS OF NEUROSYPHILIS 53 



GUMMATOUS NEUROSYPHILIS ("gumma of 
brain ")• Autopsy. 



Case 7. Mrs. Lecompte was a woman of middle age, who, 
according to the history;- given by her son, had been entirely 
well until her final illness, which began approximately two 
years before admission to Danvers Hospital. The beginning 
of her trouble seemed to be chiefly headaches, which would 
last continuously for several days, or more than a week at a 
time. These headaches lasted throughout the course of the 
disease. In the morning, on arising, she would feel very dizzy, 
but this would pass away during the day. She had had a 
number of spells of unconsciousness, lasting about fifteen 
minutes. In these attacks she would breathe heavily, there 
was frothing at the mouth, twitching of the hands, and the 
eyes would roll about. Her memory failed gradually, her dis- 
position changed and she became very irritable. Vomiting 
occurred almost every day, and at times was of a projectile 
character. She became hallucinated ; the hallucinations were 
chiefly of a visual nature. 

About four months before admission to the hospital, after 
one of her seizures, the entire right side was found to be com- 
pletely paralyzed, and she complained that It was numb. 
At this time, she had difficulty with her speech. In a few 
days, however, she was able to talk correctly again, and In a 
week she was back at work, although the right side was weak 
and awkward. She continued to grow worse, and then be- 
gan to have spells lasting several days, so that It became 
necessary to have her placed In a hospital. 

On admission to the hospital, aside from obesity, the general 
viscera showed no points of special Interest, and there was 
no evidence of any new growth outside of the nervous system. 
She was unsteady on her feet, standing with them wide apart. 
The gait was quite ataxic; the whole right side was weaker 
than the left and used more awkwardly. There was a paraly- 
sis of the right side of the face; the right angle of the mouth 



54 FORMS OF NEUROSYPHILIS 

drooped; the right eyelid could not be closed but remained 
continuously open; nor could the right side of the forehead 
be wrinkled. Vision and hearing were not affected. She 
miscalled tastes and smells ; whether this was due to aphasic 
difficulties or to cranial nerve involvement could not be 
divined. There seemed to be some difficulty in deglutition. 
The knee-jerks were markedly exaggerated; slight clonus 
was obtained but was not always present. Both pupils 
reacted well to light and distance and consensually. Sen- 
sation could not be readily tested. There was marked ataxia, 
especially with the eyes closed. The speech was thick and 
mumbling. The patient was unable to write or copy. Men- 
tally the patient was quite dull; at times, stuporous; when 
aroused, was found to be entirely disoriented. Memory al- 
most entirely absent. In general she showed herself to be 
very much confused. 

She remained practically in this condition, even gaining in 
weight, for the following two years, when suddenly one 
morning, she had an epileptic seizure, vomited, coughed a 
great deal, with bleeding from the mouth and ears, and died 
in a few hours. 

The symptoms in this case pointed to brain tumor. The 
only inconsistent thing was the long-continued life, — four 
years, — after the symptoms were observed. As she lived 
before the W. R. and spinal fluid tests were known, no light 
was gained in these ways. The post-mortem examination 
showed the patient had a Gumma of the Brain. 

The summary of the anatomical diagnoses at autopsy was: 

Decubitus. 

Lymphadenitis of the mesenteric nodes. 

Chronic fibrous peritonitis. 

Chronic fibrous myocarditis. 

Pulmonary hypostasis. 

Thrombosis of vein in right adrenal, with hemorrhage. 

Syphilitic leptomeningitis. 

Gumma of left hemisphere. 

Focal softenings in the pons. 
The anatomical description of the head (Dr. A. M. Barrett) 
is as follows : 



FORMS OF NEUROSYPHILIS 55 

The sutures in the calvarium are well outlined; 
diploe large in amount. The dura is diffusely but 
lightly adherent to the calvarium; it is very dense, 
especially over the left hemisphere. The meningeal 
arteries are thickened but not atheromatous. The 
sinuses contain a small amount of fluid blood and post- 
mortem clot. The inner surface shows nothing abnor- 
mal. There is a great flattening of the convolutions of 
the left hemisphere, which is not the case on the right 
side. Over the convexity, the pia is thin and not ab- 
normal except for some slight adhesions between the 
frontal lobes and the two lips of the Sylvian fissures. 
The pia at the base over the cisterna, pons, and medulla 
is thick, cloudy, and of a grayish gelatinous appearance. 
It is so thick that it is easily removable in a large piece. 

The surface of the left hemisphere is dry, and the 
whole brain is flabby and bulges as if from internal 
pressure. A section through the hemispheres at the 
region of the optic chiasm shows a hard, firm area in the 
left hemisphere deep down in the white substance. It 
is about 2| cm. in diameter, with a wavy border. The 
central part is of a silver-gray gelatinous-like appear- 
ance, with red spots and whitish streaks radiating from 
the centre. In the pons on the right side, in a plane pass- 
ing through the posterior corpora quadrigemina, are two 
pinhead size softenings among the pyramidal fibres. 
The ependyma of the fourth ventricle is granular. 

Microscopic examination of the tumor: The area 
evidently contains several central necrotic foci sur- 
rounded by zones of infiltration and proliferation, with 
bordering areas of nervous tissue showing secondary 
reactions. The necrotic area stains poorly. From 
the edge there are projections of reddish homogeneous 
bands, some intermixed with well-differentiated fibril- 
Ise, probably glia fibrils. The bordering zone is densely 
infiltrated with lymphoid, plasma, and a few epithelial 
cells. The nerve tissue outside of this zone is spongy 
and infiltrated with lymphoid and plasma cells. There 
are a few scattered, shrunken nerve cells. In this zone 
and in the zone of infiltration near the necrotic area, 
there are scattered cells resembling giant cells. There 
are many obliterated vessels in the area, and other ves- 
sels show many infiltrating lymphoid and plasma cells 
in the walls. The examination of the specimen stained 
by the methods for bacilli of tuberculosis, are negative. 
The growth is a classical gumma. 



56 FORMS OF NEUROSYPHILIS 



GUMMATOUS NEUROSYPHILIS (gumma of 
spinal meninges, " meningitis li3rpertrophica cervi- 
calis of Charcot ? "). Autopsy. 



Case 8. John Wyman was first seen In his thirty-sixth 
year by Dr. James J. Putnam. He denied syphilitic infection 
and stated that the first symptoms had come four months 
before. He had begun to notice a numbness of the fingers, at 
first of the right hand and shortly thereafter of the left hand. 
After a few weeks there had been difficulty in walking, and a 
few weeks later headaches, especially on the right side, devel- 
oped. Two weeks before he was first seen medically, he 
had begun to have a feeling of tightness or constriction in 
his arms. 

It appears that micturition had been impaired early, 
that is to say, a few weeks after the initial sensory disorder 
had begun. A catheter was used for a time and improvement 
followed. Shortly before consultation retention of urine 
developed again, this time associated with rectal incon- 
tinence. The feet began to feel heavy and dead. Then 
the legs began to be Increasingly weak so that the patient 
was almost bed-ridden. Vision appeared to be normal ex- 
cept that reading was followed by fatigue. The speech 
was also slow but the slowness could be attributed to fatigue. 

Notes of Dr. Putnam's physical examination are as follows : 
The patient lay In bed on the left side, without motion, and 
almost Incapable of motion. The tongue was protruded, 
and there was no paralysis of facial muscles, or of the eye 
muscles (the right pupil had been reported to be slightly 
larger than the left). There seemed to be a disinclination to 
move the head, but with some effort it could be moved, and 
without pain. The arms and hands were held rigidly in 
median positions; many movements were possible, but all 
were imperfect and of slight amplitude. The fingers were 
flexed to a moderate degree, and could not easily be straight- 
ened, and there was, in fact, a general rigidity of most of the 



FORMS OF NEUROSYPHILIS 57 

muscles of the body below the neck, and even, in some degree, 
of the neck. The immobility was so great that the general 
impression made was almost that of a patient with fracture of 
the spine in the cervical region. Even the breath, and es- 
pecially the inspiration, was imperfect. The legs were more 
freely movable than the arms, but still the motions were very 
stiff and awkward, and of slight amplitude; with effort the 
whole leg could be lifted from the bed, and flexed or extended 
with moderate force. The right leg was rather stronger than 
the left, but the left hand and arm were stronger than the 
right. The sensibility was almost absent over the hands 
and lower part of the arms, and was impaired over the entire 
head and neck, except the forehead, the middle part of the 
face, and the nose. It is interesting to compare the con- 
ditions of the sensibility here present with those seen in 
cervical syringomyelia. The sensibility of the upper part 
of the forehead was less good than of the lower part, and there 
was slight impairment even over portions of the lower jaw. 
The sensibility of the left (stronger) arm was rather more im- 
paired than that of the right arm, while on the contrary the 
sensibility of the left leg was better than that of the right 
leg, though the difference between them was not great. These 
statements apply to sensory tests by contact, heat, cold, and 
pricking. Knee-jerks were highly exaggerated, and likewise 
the wrist-jerks. All forced attempts at movements were at- 
tended by a high degree of muscular tremor, especially when 
the patient was fatigued or under emotional strain. The 
fingers especially were the seat of coarse tremor. 

The remainder of this clinical description (courteously sup- 
plied us by Dr. Putnam) may be quoted. A second ex- 
amination which Included also a few facts not given in the 
first examination was made on the following March 28, 1905. 
This report says " the ends of the fingers became numb about 
June I, 1904. Work was given up on July 3, and at that 
time the patient was walking very badly. No treatment was 
used and no satisfactory diagnosis made. In the course of 
July he improved somewhat, and during August he was able 
to ride out a little (these spontaneous improvements are of 
interest for the diagnosis). He went away from home for 



58 FORMS OF NEUROSYPHILIS 

a short time, but from the time of his return, about the last' 
of September, he grew worse rapidly, and fell into the con- 
dition above described, in which he was wholly unable to 
help himself, even to turning in bed. At times he had a 
great deal of pain in the neck and forehead. Antisyphilitic 
treatment was recommended, and for a time potassium iodid 
and other iodid preparations were given, but at first in 
relatively small doses (grs. 75 daily). Under this treatment 
the excretion of urine rose to four quarts daily as a maximum 
though sometimes the quantity was not so great." 

Under this treatment the patient began soon to improve, 
and continued doing somewhat better till about five months 
later. He became able to walk downstairs and out of doors, 
and regained considerable use of his hands. The quantity 
of urine passed became greatly increased by the use of the 
iodid. 

About the middle of March he became worse again. A 
careful examination of the sensibility showed that in general 
the condition was much the same as that previously reported. 
The iodid treatment, with perhaps some mercurial, waS; 
resumed; the potassium iodid was given in doses which were 
increased up to 850 grains daily, although this maximum dose 
was taken only for about one week. This large quantity 
gradually impaired the sense of taste for the time being, and 
blurred his vision, but otherwise did him no harm. Under 
this he Improved, so that he became able to run more or 
less, and went about freely, and attended to his business, 
though still retaining some stIfTness In his movements. 

This Improvement continued until about two years later, 
when he again had a relapse, and was seen medically once 
more. His condition at this time was still a pretty good one, 
but the movements were stiff and awkward. The bin-lodid 
of mercury was advised, which was taken In doses of 2t grain 
daily. It will be remembered that this was long before the 
days of salvarsan treatment. 

This was toward the end of June, 1907. Contrary to ex- 
pectation, there was no material gain from this treatment, 
and the patient died early In October, without being seen 
again. 



FORMS OF NEUROSYPHILIS 59 

. The autopsy was limited to the nervous system and the 
' findings were as follows (Dr. A. R. Robertson) : 

Head: Hair abundant, fair, of fine texture and 
rather curly. Scalp of medium thickness and strips 
readily from calvarium. The latter appears normal 
and upon removal is of about the normal thickness. 
It lifts readily from the dura mater, except for the 
numerous attachments of Pacchionian granulations. 

Meninges : The dura is smooth, moderately injected 
and shows no areas of thickening; it lifts readily from 
the pia-arachnoid. The pia-arachnoid shows discrete 
and in many places diffuse areas of opacity. There is a 
moderate amount of subpial clear fluid and the vessels 
are moderately injected. Over the anterior surface 
of the medulla and lower portion of the pons and largely 
confined to the right side there is a very marked thick- 
ening of the pia-arachnoid to which the dura is densely 
adherent. This thickening extends down anteriorly 
and laterally on the right side over the upper part of the 
cervical cord. The thickened meninges over the upper 
part of the medulla completely surround the right 
vertebral artery, shortly before it joins its fellow of the 
opposite side to form the basilar. Dissection of the 
arteries shows them to be patent and thin walled. 
Over the cerebrum and cerebellum the pia-arachnoid 
strips readily leaving a smooth surface. Section of 
the cerebral cortex, basal nuclei, pons and cerebellum 
show no gross lesions. The ventricles are moderately 
distended with fluid. The ependyma contains nu- 
merous small cysts. Section of the pons shows no 
lesions of the nervous tissue, but very marked thicken- 
ing of the surrounding meninges as noted above. 

Cord: Throughout the cervical and dorsal region 
the dura is quite tensely distended with an abundance 
of clear, light, straw-colored fluid. Upon snipping the 
dura this fluid escapes with a small spurt, as if under 
considerable pressure. The cord within, for the most 
part, lies free, but over the upper three or four centi- 
meters of the cervical portion it is densely adherent to 
the dura anteriorly and laterally on the right side. 
Cross sections were made through the upper three or 
four centimeters of the cord, and over this area the cord 
is constricted by very marked thickening of all the 
meninges. The meninges here average from one to 
three millimeters in thickness. On the right side and 



60 FORMS OF NEUROSYPHILIS 

somewhat anteriorly opposite the junction of the atlas 
and axis there is a single nodular, firm mass which 
on section shows a yellowish, firm center surrounded by 
very dense, pearl-gray tissue. The demarcation be- 
tween the homogeneous yellowish centre and its sur- 
rounding gray tissue is very sharp. This nodule 
measures about 0.75 to i cm. in diameter. The adjacent 
cord is deeply indented by it. Below this nodule there 
is a translucent, grayish appearance of both posterior 
sensory columns which extends downwards and di- 
minishes in intensity until it finally disappears in the 
upper dorsal region. This same appearance is well 
marked on the right outer margin of the upper cervical 
cord corresponding to the crossed pyramidal tract, and 
extends downwards diminishing in intensity until it 
disappears about the mid-dorsal region. The left 
pyramidal tract appears to be similarly but very 
slightly involved; section of the lower dorsal cord en- 
tirely negative. Microscopically, characteristic Gumma. 



It is a question whether this case is one of the group de- 
scribed in 1 87 1 by Charcot under the name of pachymenin- 
gitis cervicalis hypertrophica. Charcot did not regard his 
new disease as syphilitic, and it is very probable that syphilis 
is not responsible for all cases. Charcot, however, noted that 
his new disease was not incurable: he noted that the re- 
sulting paraplegia, although it might be very marked and 
accompanied by flexion of the leg on the thigh and although 
the paraplegia might have lasted a very long time, might 
end in recovery. Charcot thought that surgical intervention 
was necessary. He described three periods in the disease, 
the first or neuralgic (pseudo-neuralgic) was characterized by 
sharp pains in the neck and by the sensation of constriction 
in the upper part of the thorax. The second phase of the 
disease was, according to Charcot, the paralytic phase, in 
which a cervical paraplegia accompanied by muscular atrophy 
developed. Sometimes cases were found to remain in this 
paralytic phase and even to end spontaneously in cure. If 
the muscular atrophy was degenerative, then the atrophy 
was never replaced ; but, according to Charcot, some cases of 
atrophy were simple and accordingly curable. If, however. 



FORMS OF NEUROSYPHILIS 6l 

the spinal cord itself became involved in the meningeal 
inflammation, then phenomena of transverse myelitis set in 
with a spastic paraplegia and involvement of the bladder and 
rectum. Muscular atrophy never developed in the legs, at 
least in typical cases. 

Among the causes of this condition the following have 
been mentioned: cold, overexertion, alcoholism, tubercu- 
losis and syphilis. Syphilis undoubtedly plays the major 
part. Even before the days of the W. R., observers, among 
whom may be mentioned Dejerine-Tinel and Pforringer, 
discovered syphilis in nearly all sufferers from pachymenin- 
gitis cervicalis hypertrophica. 

It should be differentiated from caries of the spine and 
cord and meningeal tumors. The spinal fluid examination 
makes this somewhat easy. 

Antisyphilltic remedies are indicated, and should be tried 
even when the etiology is obscure, if only as a therapeutic 
test. 



But what have been thy answers? What but dark, 
Ambiguous, and with double sense deluding, 
Which they who asked have seldom understood, 
And, not well understood, as well not known? 

Paradise Regained, Book I, lines 434-437, 



II. THE SYSTEMATIC DIAGNOSIS OF THE 
MAIN FORMS OF NEUROSYPHILIS 



PARETIC NEUROSYPHILIS (" general paresis ") 
sometimes persistently receives the diagnosis 
NEURASTHENIA simply through omission to 
apply approved diagnostic methods. 



Case 9. Greeley Harrison, a man of 46, certainly looked 
like a neurasthenic. He wanted aid for nervous indigestion 
of years' standing, headache, insomnia, nervousness, failing 
memory, and deafness. He volunteered, in fact, that he had 
neurasthenia, and that he had been treated for this by hypo- 
phosphites. 

During the practically negative physical examination, Har- 
rison complained of headache and throbbing in the head, 
and during examination of the abdomen felt much nauseated 
and proceeded to vomit rather persistently. There were 
hemorrhoids. 

Neurological examination showed that the left pupil was 
smaller than the right, was irregular, failed to react con- 
sensually, and reacted very slowly to direct light. For the 
rest, however, the neurological examination was negative. 
On account of the nausea and vomiting, special examination 
of the gastric contents was made, but nothing abnormal 
was found. 

Mentally, it was rather striking that the patient's memory 
was quite inaccurate both for remote and for recent events. 
His school knowledge was very meagre. As for delusions, 
the only approximation thereto was the patient's continually 
dwelling upon his bodily symptoms. Emotionally, he varied 
between depression and a sanguine attitude. 

63 



64 SYSTEMATIC DIAGNOSIS 

Although there was no symptom directly suggesting syphi- 
lis in the Harrison case, the slightly abnormal pupillary 
reactions and the amnesia warranted the suspicion of syphilis. 
The blood and spinal fluid both proved positive to the W. R. ; 
the gold sol reaction was of the " paretic " type; there were 
i8 cells per cmm.; there was considerable globulin, and an 
excess of albumin. On the whole, therefore, we felt entitled 
to make the diagnosis General Paresis. Why should 
not a careful observer have considered syphilis seriously? 
Yet in our experience such cases are frequently diagnosticated 
neurasthenia, thus entailing dangerous delay in treatment 
(in this case, five years' delay). 

Going over the history of the case with still greater detail, 
we learned that for a number of years past, there had been 
symptoms of a neurological nature. For instance, five years 
before, at the age of 41, the patient had been apparently over- 
come when working near a stove, and went upstairs talking 
incoherently, but recovered shortly. Thereafter, such spells 
occurred almost every month; later, more frequently; still 
later, the attacks were associated with unconsciousness and 
amnesia. Occasionally preceding the attack there would be 
twitching of the mouth, jerking of the arms, and incoherent 
talk. Throughout these last five years, in point of fact, the 
patient had been unable to do regular work, had been given 
to much complaining, and had been far less efhcient than 
formerly. In short, it would seem that, with the improved 
technique now in the possession of medical science for the 
diagnosis of general paresis, cases like that of Harrison will be 
diagnosticated earlier and earlier. 

I. How typical is the insidious onset of symptoms in the 
case of Harrison? The onset of symptoms in neuro- 
syphilis is ordinarily considered to be sudden, and this 
statement is generally true despite the fact that after 
the diagnosis is established a number of mild prodro- 
mal symptoms can be remembered by the relatives. 
However, some cases, of which Harrison is an example, 
have an exceedingly insidious onset without sudden ac- 
cess of striking symptoms. Jojffroy and Mignot re- 
mark that with the improvement of clinical methods, 
the course of paretic neurosyphilis must now be stated 



SYSTEMATIC DIAGNOSIS 65 

to take some six or seven years for completion. In 
point of fact, there were early episodic symptoms 
(seizures almost monthly) which should not have es- 
caped medical attention. They did escape medical 
attention, however, and Harrison was wont to say 
" Why wasn't I told that my disease was syphilis five 
years ago?" 

2. Is there such a disease as syphilitic neurasthenia? Ac- 

cording to Kraepelin, syphilitic neurasthenia has been 
described as occurring shortly after infection and in 
the first stages of syphilis. There are milder and 
severer forms; the milder forms show discomfort, diffi- 
culty in thinking, irritability, insomnia, cephalic pres- 
sure, indefinite variable, uncomfortable sensations, and 
pains. The severer cases acquire anxiety, more pro- 
nounced emotional disorder, dizziness, disorder of 
consciousness, difficulty in finding the right word, 
transient palsies, pronounced sensory disorders, nausea, 
and increase of temperature. Kraepelin is in doubt 
whether there is any definite clinical picture of this sort, 
and whether there is any causal relation between the 
syphilitic infection and such symptoms as those de- 
scribed. If the effect of knowledge concerning infection 
is a merely psychic effect, then it is improper to term 
the neurasthenia in question a syphilitic neurasthenia. 
For the relation of hysteria to the acquisition of syphilis, 
see below the case of Alice Caperson (46). In point of 
fact, modern work has shown even in the primary and 
secondary stages of general syphilis more or less pro- 
nounced neurosyphilitic phenomena in the shape of 
the so-called meningitic irritation of French authors. 
(Besides the case of Caperson (46), see the case of 
Fitzgerald and the discussions under these cases.) 

3. What is the relation of the early symptoms of this case 

to the so-called preparesis of Dana? The case might 
well have been an example of Dana's preparesis. For 
a discussion of this, see Case of William Twist (13). 

4. What is the classical differential diagnosis between 

paretic neurosyphilis and neurasthenia? The testing 
of the blood by the W. R. is unconditionally necessary. 
If the W. R. is negative, the diagnosis of paretic neuro- 
syphilis is extremely improbable. (It must be borne in 
mind that a number of cases of paretic neurosyphilis 
have been shown to have a negative W. R. in the serum, 
and receive a proper diagnosis only after spinal fluid 
examination.) Next to the serum W. R. stand the 



66 ' SYSTEMATIC DIAGNOSIS 

pupillary and aphasic symptoms. In the presence of 
Argyll- Robertson pupil or even a slight speech defect, 
the diagnosis of neurasthenia must certainly be made 
with caution if at all. Kraepelin remarks: The sudden 
occurrence of neurasthenic disorders in a male of 
middle age without any evident cause therefor is al- 
ways suspicious. Yet it must be emphasized that a 
complaint of occasional dizziness, slight speech defect, 
tremor of tongue, and a moderate increase of tendon 
reflexes do not possess any marked diagnostic sig- 
nificance. Clear insight and understanding of the 
nature of the disease phenomena, a persistent search 
for recovery, reasonableness in conversation, progressive 
improvement under appropriate treatment, speak for 
neurasthenia. 

Joffroy and Mignot differentiate what they call 
preparetic neurasthenia from other neurasthenic states, 
not only on the basis of its etiology but on the basis of 
its symptoms. They also call attention to the fact that 
neurasthenia, being a pure neurosis, develops either on 
a manifestly hereditary basis or upon some physical 
injury, weakening disease, or moral shock. The pure 
neurotic suffers a great deal more than the patient who 
is destined to become a victim of paresis. The char- 
acter change in neurasthenia does not amount to that 
entire transformation of personality (even to the per- 
formance of criminal acts) that we find in paretic 
neurosyphilis; at the most, the neurasthenic shows 
minor emotional disturbances and a certain patho- 
logical egoism. The psychotherapeutic test also rather 
readily dissipates many of the neurotic, hypochondriacal 
fears and feelings. Although both pure neurasthenia 
and the paretic pseudoneurasthenia are characterized 
by sexual weakness, the sexual anaesthesia of the 
preparetic is practically always preceded by a stage 
of sexual over-excitement. These finer clinical indi- 
cations, however, fade into insignificance beside the 
data that can and should be obtained from laboratory 
tests. 
5. How exceptional is such a case as that of Harrison? We 
have in our experience seen many patients with a 
similar course and configuration of symptoms, although 
the majority of these cases in a community advanced 
enough to provide easy access to a Wassermann labo- 
ratory are now diagnosticated far earlier than was the 
case of Harrison. 



SYSTEMATIC DIAGNOSIS 67 

6. What attitude shall we take toward so-called syphilo- 
phobia? It seems to us that resort to a serum W. R. 
is indicated, both from the standpoint of the community 
and still more importantly from the standpoint of the 
patient. We are even inclined to suggest for a case of 
persistent syphilophobia, when the serum W. R. has 
proved negative, a lumbar puncture. Syphilophobia 
must be considered, not as a syphilitic psychosis, but 
as a phobia to be classified among the psychoneuroses. 
It becomes a difficult question to decide at times 
whether a patient who has had syphilis, has had a 
considerable course of treatment and shows the symp- 
toms of a syphilophobiac should be further treated for 
syphilis or merely for his phobia. We have seen re- 
cently such a patient who gave a certain history of 
syphilis and who was greatly disturbed lest he should 
be developing paresis. This fear bothered him greatly. 
Examination showed irregular pupils, but no other 
signs of syphilis. The W. R. in blood and spinal fluid 
was negative as were the other spinal fluid tests. It 
was considered wise to treat him only for his phobia 
and under this treatment he was given some relief. 



68 SYSTEMATIC DIAGNOSIS 



PARETIC NEUROSYPHILIS (" general paresis ") 
may look precisely like MANIC-DEPRESSIVE 
PSYCHOSIS. 



Case 10. The mental picture in Lyman Agnew, an archi- 
tect, 58 years of age, was wholly characteristic of manic- 
depressive psychosis. In the first place, there had been (at 
55) a previous attack of depression, lasting a few months, 
from which Agnew had completely recovered. He had 
remained entirely well up to four months before consultation. 
(Manic-depressive psychosis is, at least in a majority of 
cases, hereditary. There had been mental disorder in one 
maternal cousin, and mental impairment in the patient's 
mother some time before her death from cerebral hemorrhage. 
There was no other report of mental disease in the family.) 

It appears that in the interval between attacks, Agnew 
had been working very hard and had been fairly successful 
in paying off a mortgage on his house. A marked elation, 
somewhat natural, followed this success and continued to an 
abnormal degree. Agnew labored under considerable ex- 
citement, was over-fussy, and at times showed a flight of 
ideas. His mania or hypomania gradually diminished and 
depression set in, in which depression he arrived for consulta- 
tion. He had marked ideas of self-accusation, was emotion- 
ally unstable, wept much, and showed a characteristic 
retardation of activities and unrest. 

Physically, there was no neurological disorder. The patient 
appeared rather under-nourished. The heart borders lay 
2 cm. to the right and at ii| cm. to the left of the mid- 
sternal line. The aortic second sound was very loud. There 
was a moderate radial arteriosclerosis. Systolic blood pres- 
sure was 210, diastolic 155. 

The high blood pressure suggested nephritis, possibly of 
arteriosclerotic origin, but urine examination and blood- 
nitrogen tests yielded no evidence of kidney disease. More- 
over, it is our experience that a manic-depressive psychosis 



SYSTEMATIC DIAGNOSIS 69 

in persons past middle life is not infrequently complicated 
by high blood pressure. In point of fact, some authors in- 
sist upon a relation between manic-depressive psychosis and 
the arteriosclerosis which rather frequently sets in in this 
disease. 

Routine examination of the blood serum, however, yielded 
a positive W. R. Following the approved rule of making an 
examination of the spinal fluid in all mental cases having a 
positive serum W. R. , we proceeded to lumbar puncture. The 
fluid was clear and contained 35 cells per cmm,, the albumin 
was in excess, and there was a positive globulin reaction. 
The gold sol reaction was of the " paretic " type; the W. R. 
was strongly positive. 

On this basis, it seems worth while to consider the diag- 
nosis of General Paresis or that of some form of non- 
paretic neurosyphilis. The former is the diagnosis which we 
prefer. 

1. What is the classical differential diagnosis between manic- 

depressive psychosis and neurosyphilis? The labora- 
tory tests have naturally supplanted the older purely 
clinical methods of differential diagnosis. The diffi- 
culties lodge, in the first instance, in depressive states. 
It would appear to be impossible on purely clinical 
grounds in certain cases to tell the depression of neuro- 
syphilis from the depression of manic-depressive psy- 
chosis, since the slightly greater interest in the outer 
world taken by manic-depressive patients and their 
greater responsiveness to diagnostic threats (suggestion 
that patient is to be pinched or cut) are of no special 
value in the individual case. Identical considerations 
hold for the maniacal phases of manic-depressive psy- 
chosis, for these maniacal phases may even develop 
delusions (Kraepelin) of precisely the same nature as the 
characteristic expansive delusions of the excited paretic. 

2. If the clinical symptoms are insufficient in differential 

diagnosis, are not the pupillary signs and the speech 
defect of greater value? They are of value if present, 
but as in the case of Agnew, the victim of neurosyphilis 
may show no pupillary or speech disorder. Instances 
are familiar, also, in which the pupillary and speech 
signs are absent in very advanced cases of non-paretic 
or even of paretic neurosyphilis. 



70 SYSTEMATIC DIAGNOSIS 

3. Would not a circular course or recurrence of attacks 

be decisive for manic-depressive psychosis? Paretic 
neurosyphilis sometimes exhibits the same circular 
or recurrent course. We conclude that neither the 
clinical symptoms, the classical pupillary and speech 
signs, nor the ups and downs of a particular disease, are 
at all decisive as between manic-depressive psychosis 
and paretic neurosyphilis. Resort must be had to 
laboratory tests. 

4. What is the significance of the high blood pressure in 

paretic neurosyphilis? Work from our laboratory 
(Southard and Canavan) has shown plasma cells in 
the kidneys in 17 out of 30 paretics (56%), and in 
16 of these 17 paretics with renal plasmocytosis, the 
plasma cells were found in the periglomerular region. 
What the relation of these findings may be to heightened 
blood pressure is as yet unknown. The severe syphi- 
litic involvement of the aorta so characteristic in paretic 
neurosyphilis, as in other forms, may possibly have a 
bearing on blood pressure. 



SYSTEMATIC DIAGNOSIS 7 1 



A POSITIVE SERUM WASSERMANN REAC- 
TION associated with mental symptoms (even 
with grandiosity) does NOT prove the EXIST- 
ENCE OF PARETIC NEUROSYPHILIS ("gen- 
eral paresis "). 



Case II. Juliette Lachine came to a general hospital 
with pain in the right upper quadrant of the abdomen, wherein 
was found an enlarged liver. This liver was regarded as 
syphilitic on the ground that the patient had a positive serum 
W. R. and that her two elder children were clearly suffering 
from congenital syphilis. The liver mass was promptly re- 
duced by antlsyphilitic treatment of the classical sort. 
When, however, the patient was given an Injection of salvar- 
san, she shortly began to develop marked mental symptoms, 
whereupon she was removed to the Psychopathic Hospital. 

The mental picture at the Psychopathic Hospital was as 
follows: Lack of orientation for time, marked distractlbility 
of attention, with a certain jumping from one subject to 
another, delusions of a religious nature, claims of wonderful 
powers possessed by the patient, moods variable, though as a 
rule of a euphoric and elated nature, with laughing and sing- 
ing. The activity seemed to be of a mental rather than a 
peripheral nature. The patient did not regard herself as 
mentally abnormal. The liver was still 4 cm. below the costal 
margin in the nipple line. We found the W. R. to be positive 
in the serum but negative in the spinal fluid. In fact, the 
spinal fluid was entirely negative. 

So far as we are aware the picture presented by this case 
is one of Manic-depressive Psychosis. We regard the dis- 
ease as merely complicating the syphilis, although it Is en- 
tirely possible that some visceral condition incidental to the 
syphilis might be proved (in a higher stage of psychiatric 
science) to have produced the mania. 

In any event, the patient quite recovered from her mental 
symptoms in a month. She was then able to tell us of a 



72 SYSTEMATIC DIAGNOSIS 

previous attack of depression some 12 years previously, 
namely, at the age of 26. It apears that she had at that time 
been committed to a hospital for the insane. 

1. In this case, in which the diagnosis of manic-depressive 

pyschosis and not paretic neurosyphilis was made, 
are we sure that the symptoms that we term manic-de- 
pressive psychosis were not actually produced by 
syphilotoxins? In other words, in the absence of 
spinal fluid signs of inflammation or chemical change, 
might it not be possible for generalized syphilis out- 
side the nervous system to produce manic-depressive 
symptoms? There is so far in the literature no experi- 
mental or other evidence of syphilotoxins. The ex- 
istence of products and substances permitting the W. R. 
and the gold sol reaction is not of course evidence of 
syphilotoxins. Although there is no evidence of soluble 
syphilotoxins, it is thought that in the so-called Jarisch- 
Herxheimer reaction (the intensification of clinical 
symptoms after salvarsan injection) effects may be 
due to the liberation of products from the killed bodies 
of spirochetes. Such endotoxins are not here in 
question. 

2. Is visceral syphilis, such as gumma of the liver, able to 

produce characteristic syphilitic reactions in the spinal 
fluid? We have had an autopsied case in which there 
was a " paretic " gold sol reaction of the fluid (though 
without other signs). The autopsy showed gummata 
of the liver. However, the finer anatomy of the ner- 
vous system showed a mild but definite meningo-en- 
cephalitic process, which was doubtless responsible 
for the gold sol reaction. 

3. What is the value of grandiose ideas? Ballet distin- 

guishes two groups of grandiose ideas: {a) ideas of self- 
satisfaction, including ideas concerning extraordinary 
capacity, strength, power, and wealth on the part 
of the patient; and {h) ideas of ambition; the latter 
being of a more exact, constant, uniform and systema- 
tizing nature. The more vague and less systematized 
ideas of self-satisfaction rest in a phase of contentedness 
and optimism; the more definite ideas of pride and 
ambition are responsible for striking transformations 
of personality. General paresis shows, according to 
Ballet, these Ideas of self-satisfaction in their most 
developed form. A certain variability, absurdity, in- 



SYSTEMATIC DIAGNOSIS 73 

coherence, and contradictorlness characterize these 
ideas and the patient has Httle or no insight into their 
nature. When such ideas occur at the outset of the 
disease, they naturally may be of medico-legal interest. 
Cotard explains these ideas of megalomania on the part 
of paretics on the ground that they are essentially motor 
or will disorders and rest upon a sort of hyperbulia, 
exhibiting itself in exuberant activity. Regis has 
thought that the delusional generosity and liberality 
of the paretic, and his willingness to lend his wealth 
and talents to social progress, is helpful for diagnosis 
when contrasted with the more personal egoism of the 
victim of manic-depressive psychosis. The self-satis- 
faction of the manic-depressive patient often does not 
reach a delusional stage, but remains a mere feeling of 
pathological well-being or euphoria. The maniacal 
patient may compare himself with some great man but 
he does not identify himself with him. It must be 
remembered that these ideas of self-satisfaction occur 
also in alcoholism, but according to Ballet they occur 
only in the dementing phase of chronic alcoholism, 
and have no special diagnostic value. They may be 
a clinical stumbling-block for a time in the cases of 
alcoholic pseudoparesls. As for the ideas of am- 
bition in which the patients believe themselves to be 
princes, emperors, divine messengers, and the like, these 
are less characteristic of paretic neurosyphilis than of 
delusional psychoses of a non-syphilitic nature. At 
all events, such ideas if definite, of long standing, 
and systematized by the patient to form a thorough- 
going portion of his life, are not characteristic of neuro- 
syphilis. The victim of paretic neurosyphilis can as a 
rule be persuaded out of his delusions, at least for 
the time being. These distinctions, it must be added, 
are hardly of value in the early cases of any of the 
psychoses In question, and cannot be made as a rule In 
either private or psychopathic hospital practice. Typi- 
cal examples of grandiosity, although not so frequent 
as might be thought from textbooks, are always on dis- 
play in institutions for the chronic insane. 



74 SYSTEMATIC DIAGNOSIS 



PARETIC NEUROSYPHILIS ("general pare- 
sis ") may look precisely like DEMENTIA 
PRAECOX. Autopsy. 



Case 12. Henry Phillips remains a striking case in the 
memory of those who knew him and his medical findings. 
Phillips came to the hospital voluntarily at 42 years of age 
from the bank where he worked as a clerk; he came at the 
suggestion of his employer. It seems that he had been 
annoying his associates because he had fallen into a habit of 
continually scratching himself. Phillips was entirely sure 
that he was the victim of what he called the " Scotch itch," 
and explained ofT-hand that this itch had been put upon him 
by the Free Masons as a matter of revenge because he would 
not join their order. He said once, for example: "At 
times I feel like raising Hell ; then I get a psychic intimation ; 
and then I get to using a foot-rule on my back and to slapping 
my face." He explained this psychic intimation as coming 
from the order of Scottish Rites. Another example of talk is 
as follows: " My father is a fighting man; that is part of it. 
They mean to throw me down. I am through now trying 
for membership in the Free Masons. They have good cause, 
they must fight. They do not want me for some personal 
matters. I can go just so far in agreeing and seconding their 
advances, but in the end it fails. I have no strength nor 
endurance." 

Aside from these delusions, there was little abnormality 
to be found, though his recollection for minor events of the 
immediate present was inaccurate. He was rather abnor- 
mally impulsive, gesticulating a good deal while talking, 
and was of the appearance that the laity call " nervous.** 
It appears that he had always been peculiar, subject to 
violent fits of temper, in which fits he might throw things at 
other members of the family. He always had pronounced 
likes and dislikes which he never concealed. He had never 
had friends, had always been secretive; and he was often 



SYSTEMATIC DIAGNOSIS 75 

termed a great student. For some five years he had been 
studying Japanese from time to time, associating himself 
with a Japanese. 

It never does to jump at the diagnosis dementia praecox. 
However, the picture seemed characteristic enough for the 
paranoid form of this disease. Physically, Phillips had 
no particular abnormality; the knee-jerks were a little lively, 
and the pupils reacted a little sluggishly. However, the 
routine W. R. of the serum proved to be positive. Exami- 
nation of the spinal fluid was resorted to, — as in all cases 
with a positive serum W. R. — and it also proved to be 
positive and strongly so; the globulin and albumin were in- 
creased, and there was a pleocytosis. A diagnosis of neuro- 
syphilis was hardly avoidable. Phillips later admitted a 
chancre, which he claimed was located on the mucous mem- 
brane of the cheek and acquired by using the same utensils 
as his Japanese friend, which friend, he stated, had active 
syphilis. 

Antisyphilitic treatment of considerable intensiveness 
was begun, with intravenous injections of salvarsan and 
intraspinous injections of salvarsanized serum, but the 
patient grew steadily worse. His mental symptoms became 
more marked, although not especially characteristic of general 
paresis. Neurologically, he did develop signs more sugges- 
tive of general paresis, and i8 months later died. 

The autopsy showed features of General Paresis. It is 
not necessary to enter into the question of the details of his- 
tological correlation at this time. 



1. What conclusion can be drawn from lively knee-jerks? 

Lively knee-jerks are of very little significance. Not 
only certain neurosyphilitlcs but also a variety of neu- 
rotic persons, victims of dementia praecox and hysteria, 
are very prone to have active tendon reflexes. Of 
course, extreme degrees of exaggeration are of import- 
ance, and especially an association of the hyperreflexia 
with the Bablnski reaction, the Gordon, or Oppenheim 
reflexes, ankle clonus, and the like. 

2. Is there any special or differentiating factor in an extra- 

genital chancre as against a genital chancre? Prob- 



76 SYSTEMATIC DIAGNOSIS 

ably this question should be answered in the negative. 
Some have claimed that chancres draining by lymphatic 
channels of the head are more likely to lead to 
cerebral syphilis. This idea cannot be said to be 
established. 
3. Is there any significance in the story, if true, that 
Phillips acquired his syphilis from a Mongolian? It 
seems to be fairly well established that syphilis of the 
nervous system is extremely rare in China and Japan, 
whereas bone syphilis is very frequent there. It has 
been held that this has to do (a) with strains of 
spirochetes, (b) with the state of civilization, or (c) 
with the degree of " syphillzation." Apparently when 
a race is first infected with syphilis the lesions are 
chiefly of the cutaneous and osseous systems; only in 
later generations the vascular and nervous systems 
suffer. However, involvement of the nervous systems 
of Mongolians resident in this country is no rarity, a 
point possibly in favor of the theory of special strains 
affecting the nervous system as prevalent in western 
countries. Little or nothing is known as to the effect 
of transmission from one race to another, as from 
Mongolian to Caucasian in Phillips' story. 



SYSTEMATIC DIAGNOSIS 77 



NEUROSYPHILIS is NOT to be entirely ruled out 
by a negative serum Wassermann Reaction; for 
the fluid Wassermann Reaction may be positive. 



Case 13. William Twist is a case of note in the matter 
of the so-called preparetic period (the idea of Charles L. 
Dana which was scoffed at when first proposed by him iniQio). 
The patient, a very successful traveling salesman, 35 years 
of age, was admitted to the Psychopathic Hospital showing 
a typical picture of general paresis. 

Thus, mentally, the patient showed elation, grandiosity 
(millions of dollars to give away), intellectual weakness, 
disorder of memory, lack of judgment, rambling talk, speech 
defect, omission of letters in writing and spelling. 

Neurologically, there was tremor of the lips, sllghti^Irregu- 
larity of the pupils, which however reacted well, and lively 
knee-jerks. 

Mr. Twist had sought advice at our out-patient department 
in his thirty-third year. The records show that at that 
time he was somewhat depressed, and his speech was even 
then, according to his own statement, stammering. However, 
we found the W. R. at that time to be negative in the blood 
serum. It appeared that his mother had died of consump- 
tion; his father was said to have committed suicide. A 
brother had once recovered from an attack of depression, 
presumably an attack of manic-depressive psychosis. Ac- 
cordingly, we thought at the time that the case was probably 
one of manic-depressive psychosis. Moreover, our routine 
serum W. R. failed to indicate any syphilitic process. As for 
the so-called stammering of speech, this appeared to be a 
matter of the patient's own recollection rather than of our 
observation. In any event, the patient had gone into the 
country and appears to have entirely recovered ; falHng, again, 
however, into mental difficulties after a short period, and 
finally arriving at the hospital in the above-mentioned classi- 
cal condition. 



78 SYSTEMATIC DIAGNOSIS 

The W. R. in the blood serum proved again negative. 
The test was repeated a number of times ; also, after salvar- 
san had been given. The salvarsan did not act provoca- 
tively, and the blood serum has remained consistently nega- 
tive. 

In cases of syphilis the W. R. is at times negative. Swift 
claims that in such cases an injection of salvarsan will often 
produce a positive W. R. if the blood is tested on several days 
following the injection. 

The spinal fluid, however, did show a positive W. R. as 
well as a gold sol reaction of a " paretic " type. There were 
at the first examination 194 cells per cmm., there was a mod- 
erate excess of albumin, and a positive globulin test. In 
short, there was no question of any other diagnosis than 
General Paresis. 

1. How can the negative W. R. of the blood serum be ex- 

plained? It is difficult or impossible to explain this. 
Figures differ as to the percentage of cases of general 
paresis with negative blood serum; perhaps 3 to 5% of 
these cases yield a negative serum W. R. 

It is important to note the long preparetic period : at 
least a year and a half. Could our diagnostic methods 
be sharpened a trifle, such cases as these could be ob- 
tained early in this preparetic period and it might 
then be safe to promise good therapeutic results. 

2. What is the nature of the preparesis of Dana? When 

Dana's brief paper on preparesis was written, there 
was of course hardly any idea that cases of paretic 
neurosyphilis could be cured or would recover, except 
possibly vanishingly few cUriosa about which there 
would always rage a diagnostic question. Accordingly, 
Dana, having found certain cases that seemed to him 
to have early signs of paresis but had apparently been 
cured by treatment, proposed to call them cases of 
preparesis. His idea was that he would thereby not 
offend those who held that general paresis was theo- 
retically a fatal disease. With modern work and the 
display of more and more atypical cases of neuro- 
syphilis, and the observation of relatively numerous 
cures or remissions under treatment, the designation 
of preparesis for a separate entity, or even for a sub- 
form of neurosyphilis, becomes superfluous. 



SYSTEMATIC DIAGNOSIS 79 

3. What is the percentage of cases of paretic neurosyphilis 

that show a negative serum W. R.? Among the best 
figures are those of Mil Her, who found that of 386 ex- 
amples of paretic neurosyphilis, 379 showed all reac- 
tions positive, or 98.5%. 

4. What is the meaning and value of the so-called pro- 

vocative salvarsan injection? In practice, there may 
be a series of negative W. R.'s in the blood serum 
before a positive reaction is finally obtained, owing to 
technical difficulties or biological peculiarities. Where 
intensive work is being done upon the neurosyphilis 
problem, it is beyond question desirable to make the 
W. R. test upon at least three separate samples of blood 
drawn at intervals, for the second or third test may 
prove positive. This situation makes the interpretation 
of the so-called provocative salvarsan injection exceed- 
ingly doubtful; that is, the reaction might have been 
positive on repetition without the injection of salvarsan. 
The present case, as above stated, failed to yield a serum 
W. R. even after repeated tests and the " provocative." 

5. What is the significance of the irregular pupils in this 

group? Paretic neurosyphilis shows inequality of the 
pupils in a high per cent of cases. Irregularity of out- 
line of the pupils is commonly thought to be an im- 
portant sign and to suggest neurosyphilis. It is true 
that many cases of pupillary irregularity are syphilitic, 
but the sign is of little or no differential value since 
congenital malformations and relics of old injuries and 
adhesions may produce effects identical with those of 
neurosyphilis. 



8o SYSTEMATIC DIAGNOSIS 



DIFFUSE (that is, meningovasculoparenchyma- 
tous*) NEUROSYPHILIS is typically associated 
with six positive tests (serum Wassermann reaction, 
fluid Wassermann reaction, spinal fluid gold sol 
reaction, pleocytosis, positive globulin, excessive 
albumin) ; but one or more, and frequently sev- 
eral, of these tests are likely to run mild as 
compared with the tests in PARETIC NEURO- 
SYPHILIS ("general paresis"). [The clinical course 
of the diffuse (and especially the meningovascular) 
cases is likely to be protracted, with a good prog- 
nosis as to life (barring fatal vascular insults). 



Case 14. We shall present the case of John Jackson, a 
surveyor, 31 years of age, suffering from a left hemiplegia, 
with this in mind: To exhibit difficulties in diagnosis in the 
presence of an embarrassment of symptomatic riches. 

The patient arrived at the hospital, in the first place, be- 
cause he had been threatening a woman who lived next door 
to him. He believed that this neighbor had been talking 
about him and circulating reports against him. Excited by 
these ideas, he had threatened to cut her throat. 

Now the occurrence of hemiplegia in adult life before the 
approach of senium is always suspicious of syphilis, and this 
suspicion we naturally entertained from the beginning. 
However, there was upon the scalp a crooked linear furrow 
about six inches long, running from the vertex to the right 
parietal eminence. Another furrow about an inch long 
was present upon the forehead. These furrows appeared to 
be of a bony nature and were not tender. There was evi- 
dence of an old decompression operation on the right side of 
the head; there were also large scars on both sides of the 

* Proof of marked parenchymatous lesions must hang on 
post-mortem data; the inference here as to the presence of 
parenchymatous lesions is a clinical inference. 




Station in syphilitic hemiplegia. 
Syphilitic pigmentation of skin. 



SYSTEMATIC DIAGNOSIS 8 1 

neck, evidently the result of old operations; and there were 
numerous palpable glands — the largest about the size of 
a lima bean — all firm and not tender. 

It seems that at the age of eight, according to the patient's 
mother, Jackson had received a head injury and had re- 
mained unconscious for three weeks. Upon recovery, he 
had to relearn both to walk and to talk; however, he was 
able to begin school where he left off. He became more ner- 
vous and irritable after the accident than previously. Noth- 
ing further had developed until, at about 25 years of age, a 
tubercle was discovered in his eye (the right pupil was 
smaller than the left, reacting more slowly; right iris bound 
down by adhesions, with white opacity of anterior chamber). 
For two years, 25 to 27, the patient was under medical 
treatment for tuberculosis, and at the conclusion of this 
period numerous glands were removed from the neck and 
diagnosticated tuberculous. However, the neck did not 
heal and he carried bandages upon it for two years. 

At 28, the patient's mother described the occurrence of 
a slight shock, with head retraction, for a minute or two, 
and inability to speak. Thereafter there had been five or six 
similar attacks, less severe, and without loss of speech. The 
attacks were never accompanied by convulsive movements. 
Then occurred a paralytic stroke, leaving the patient with a 
left hemiplegia, which had somewhat improved. Mentally, 
the patient had gone down hill, becoming less alert and more 
apathetic, and to some extent amnestic. One had to con- 
sider, accordingly, the somewhat doubtful possibility of 
post-traumatic and post-operative conditions, and the ques- 
tion of tuberculosis (possibly errors in diagnosis; the lungs 
showed no evidence of tuberculosis) . 

Physically, the signs of a left hemiplegia were appropriate. 
Spasticity on the left side was found; there were Babinski, 
Gordon, Oppenheim reflexes and ankle clonus on the left 
side (all absent on the right). Speech defect was present. 
Mentally, aside from the delusions noted at the beginning of 
our analysis, a striking feature was the patient's childishness. 
While reciting delusions, the patient was overactive and 
evinced a somewhat childish interest. Arithmetically, Jack- 



82 



SYSTEMATIC DIAGNOSIS 



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SYSTEMATIC DIAGNOSIS 83 

son had preserved a fair ability but his apathy and lack of 
interest interfered with tests, and possibly also with the 
exercise of memory. As above noted, we were compelled 
to maintain the suspicion of syphilis throughout despite 
the attractive hypotheses of traumatic and post-decom- 
pressive effects and cerebral tuberculosis. A history of the 
acquisition of syphilis an unknown number of years before 
admission entered to strengthen the suspicion of the syphilitic 
nature of the mental symptoms. 

The W. R. proved positive in blood and spinal fluid. The 
gold sol reaction was of the syphilitic type; 37 cells were 
found per cmm. ; there was a slight amount of globulin and a 
slight excess of albumin. 

We made a diagnosis of Cerebrospinal Syphilis rather 
than general paresis on account of, first, the slow course of 
the disease; second, the vascular type of the cerebral insult, 
hardly typical of paresis; and third, the mild spinal fluid 
reaction. Treatment will hardly cure the hemiplegia, at 
least so far as restoration of cerebral tissues lost in the insult 
is concerned. We were perhaps entitled to consider that, as 
in the cases of Petrofski (17), O'Neil (19), Robinson (45), 
the meningitic process could be arrested. Unfortunately, 
our treatment of 20 injections of salvarsan over a period 
of 10 weeks, followed by a number of months of bi-weekly 
injections of mercury salicylate, proved incapable of making 
any change in the mental and physical picture or in the 
laboratory findings. 

I. Can we explain the apparently poor reaction to treatment of 
the cerebrospinal syphilis in the case of Jackson by 
supposing a more deep-seated involvement than the 
meningovascular involvement indicated by the hemi- 
plegia and the signs in the fluid? Autopsied cases in 
our experience show focal parenchymatous involve- 
ments that have not caused obvious clinical symptoms 
at any time during the course of the disease. These 
symptomatically silent lesions may have been present. 
'2. What is the comparative prognostic value of seizures 
in paretic neurosyphilis and in such a meningovascular 
case as that of Jackson? Paretic seizures are often 
and indeed characteristically recovered from. More- 



84 SYSTEMATIC DIAGNOSIS 

over, autopsies in paretic neurosyphilis characteris- 
tically show no gross focal destructive lesions to cor- 
respond with the seizures. The paretic seizures are 
apparently more irritative than paralytic. However, 
the seizures of the meningovascular group of neuro- 
syphilis are also, though less commonly, recovered from, 
so that the differential diagnosis on the basis of the 
outcome of seizures is not safe. Rarely paretic neuro- 
syphilis itself also develops seizures from which no 
recovery is made. 
3. What is the relation of neuropathic heredity to neuro- 
syphilis? The family history of John Jackson is un- 
doubtedly poor, since his father died of diabetes and 
a paternal uncle was insane; and on the mother's side, 
the grandmother died of tuberculosis and an aunt died 
insane. This general question was more interesting in 
the days before the syphilitic nature of general paresis 
and of allied diseases was known. However, we may 
still hold perhaps that not only syphilis but also vari- 
ous intoxications, especially alcoholism, do flourish upon 
a neuropathic soil. This question, like that of Krafft- 
Ebing's celebrated claim of the relation between syphil- 
ization and civilization, needs revision in the light of 
more extensive applications of the W. R. in larger 
and larger groups of persons under various community 
conditions. 



SYSTEMATIC DIAGNOSIS 



85 



The SIX TESTS (serum Wassermann reaction, 
fluid Wassermann reaction, pleocytosis, gold sol 
reaction, globulin, excess albumin) are likely to 
run STRONGER in PARETIC NEUROSYPHILIS 
(" general paresis ") than in DIFFUSE (especially 
meningovascular) NEUROSYPHILIS; in particu- 
lar, the gold sol reaction is likely to prove 
** paretic " rather than " syphilitic." The clinical 
course of paretic neurosyphilis (" general paresis ") 
is likely to terminate in death within a few 
years. 



Case 15. Pietro Martiro was a well-developed and nour- 
ished man, 30 years of age, who had been doing erratic things 
and acting peculiarly for a few weeks before entering the hos- 
pital. In the hospital, Martiro proved to be very excitable 
and given to violence. He had marked delusions of grandeur, 
saying he was worth many millions of dollars, was the greatest 
singer in the world, the greatest athlete in the world, and 
the like. 

Physically, there was no disorder except overactivity 
of some reflexes. The diagnosis of General Paresis offered 
no difficulties, and it was confirmed by the laboratory tests 
(positive serum and fluid W. R., " paretic " gold sol reaction, 
42 cells per cmm., an excess of albumin, and a positive 
globulin test) . 

Treatment: The perfect physique of this case and the 
extremely brief clinical duration (a few weeks) would naturally 
suggest a probably favorable outcome. However, cases 
with marked delusions of grandeur have very frequently 
proved to be cases with extensive brain tissue loss as shown 
in certain studies with Dan vers material. 

In any event, the treatment in this case proved unavailing. 
Enormous doses of salvarsan, twice a week, aided by mercury 
and potassium lodid, were given. Although other cases had 
been helped by such intensive treatment, Martiro went 



86 SYSTEMATIC DIAGNOSIS 



PARETIC NEUROSYPHILIS (GENERAL PARESIS) 

PHYSICAL SYMPTOMS 

EARLY HEADACHE 

VISUAL DISORDER 

HYPALGESIA 

ADIADOCHOKINESIS 

ATAXIA 

NASOLABIAL FLATTENING 

VOCAL CHANGE 

SPEECH disorder: 

WRITING DISORDER 

LOSS OF MANUAL DEXTERITY 

PUPILLARY CHANGES 

REFLEX CHANGES 

SEIZURES 

LATE: PARALYSIS, CONTRACTURE 



Chart 9 



SYSTEMATIC DIAGNOSIS 87 



PARETIC NEUROSYPHILIS (GENERAL PARESIS) 

MENTAL SYMPTOMS 

INTAKE IMPAIRED 

CONSCIOUSNESS CLOUDED 

FATIGUABILITY INCREASED 

HALLUCINOSIS RARE 

AMNESIA — RECENT! CHRONOLOGY AND STORAGE 
IMPAIRED. FABULATION 

OVERSUGGESTIBILITY 

JUDGMENT IMPAIRED. 

FANTASTIC DELUSIONS 

INSIGHT INTO ILLNESS NIL 

EARLY IRRITABILITY OR HEBETUDE 

QUICK SHIFTING EMOTION 

CHARACTER CHANGE 

CONDUCT SLUMP 



Chart 10 



88 SYSTEMATIC DIAGNOSIS 

steadily downhill, nor was there the slightest diminution in 
the intensity of any of the spinal fluid reactions. After 50 
injections of salvarsan over a period of 30 weeks without im- 
provement, treatment was discontinued. A few months 
later, the patient died. 



1. What is the duration of paretic neurosyphilis (" general 

paresis")? If we omit the doubtful, early, and pro- 
dromal stages and count the beginning of the disease 
with the occurrence of definite symptoms, we find 
(Kraepelin) that almost half the patients with pro- 
nounced paretic signs die within the first two years of 
their disease. Kraepelin's observations upon 244 cases 
are as follows : 

Year: i 2 3 4 56789 10 14 

Cases: 51 63 52 41 22 4 5 2 2 i i 

The average duration of the disease in months has 
been calculated as varying from 24 to 32 months. 
Juvenile paresis runs a slower and more insidious 
course. The duration of paresis, according to many 
observers, diminishes with the increasing age of the 
patient. It is now held that a combination of tabes 
with paresis does not prolong the duration of the 
paresis. As noted above in the discussion of Case 
Harrison (9), our conceptions of the characteristic 
duration of paretic neurosyphilis must alter with the 
increase of our knowledge due to the early application 
of laboratory tests. 

2. What is the significance of the term general paresis? 

The case of Martiro is, of course, a good instance to 
show that the term is sometimes a misnomer. The 
characteristic generalized motor incapacity denoted 
by the term general paresis is shown in patients in the 
institutions for the chronic insane in their last few 
months of life. The term paresis is perhaps to be 
preferred to the term paralysis because the paralysis is 
not complete but partial; but perhaps the best reason, 
is that the word paresis is a shorter word. When the 
mental side is to be emphasized, the term paralytic 
dementia is employed. In this book we have used the 
term paretic neurosyphilis to mean a more precise 
statement of the etiology of general paresis (general 
paralysis, paralytic dementia). The lay term, softening 




Euphoria in paretic neurosyphilis ("general paresis"). The head, 
arms and trunk were shaking with mirth; hence, the indistinct out- 
lines of the photograph. 



SYSTEMATIC DIAGNOSIS 89 

of the brain, like the terms metasyphilis and parasypMlis 
is in the present phase of our knowledge to be eschewed. 
If this fatal case be typical of general paresis (for more 
favorable results, see Part V), what is the toll of deaths 
from this disease in the community at large? A striking 
statement may be quoted from Dr. Thomas W. Salmon's 
"Analysis of General Paralysis as a Public Health 
Problem:" 

" With the information in our possession at the 
present time, we are able to state that not fewer than 
1000 persons in whom general paralysis is recognized 
die in New York State every year. Let us compare 
this with the lives lost from some other important 
preventable diseases. It means that one in nine of 
the 6909 men who died between the ages of 40 and 60 
in New York last year died from recognized general: 
paralysis and that one in thirty of the 5299 women 
who died in the same age-period died from this disease. 
" The number of deaths from general paralysis in 
New York last year about equalled the number of 
deaths from typhoid fever. The following table gives 
the number of deaths due to the ten most impor- 
tant specific infectious diseases. Of course, deaths in 
measles, typhoid fever and scarlet fever will be found 
also under the names of some of the complications of 
these diseases, but it should be remembered that 
these primary diseases are not invariably fatal as general 
paralysis is. Many of the patients with measles who 
died from bronchopneumonia would have recovered 
but for this complication, while the paretics with 
bronchopneumonia would have died even if this com- 
plication had not arisen. No attempt is being made to 
compare the prevalence of general paralysis with that 
of other diseases — we are trying only to estimate its 
share in the mortality. 

" I. Tuberculosis (all forms) 16,133 

2. Pneumonia 9,302 

3. Bronchopneumonia 7,217 

4. Diphtheria and croup 1,854 

5. Influenza 1,381 

6. Measles 1,071 

7. Typhoid Fever 1,018 

General paralysis {recognized) . . 1,000 

8. Scarlet fever 837 

9. Whooping cough 818 

10. Syphilis 782 " 



90 SYSTEMATIC DIAGNOSIS 



PARETIC NEUROSYPHILIS (GENERAL PARESIS) 

CHARACTERISTICS 

AMNESIA 

QUICK SHIFTING EMOTIONS 

CHARACTER CHANGE 

CONDUCT SLUMP 

NERVOUS DISORDERS 

SPEECH DISORDERS 

PUPILLARY CHANGES 

REFLEX CHANGES 

SEIZURES 

CEREBROSPINAL FLUID PICTURE 



Chart ii 



SYSTEMATIC DIAGNOSIS 9 1 





SYPnnJTIC PSYCHOSES 




SYPHILITIC 


NEURASTHENIA 






GUMMA 








SYPHILITIC 


PSEUDOPARESIS 






APOPLECTIC 


CEREBRAL SYPHILIS 






SYPHILITIC 


EPILEPSY 






SYPHILITIC 


PARANOIA 






TABETIC PSYCHOSIS 






HEREDITARY 






PARESIS 












Kraepelin, 


1910 






Chart 12 



92 SYSTEMATIC DIAGNOSIS 



TABOPARETIC NEUROSYPHILIS ("tabopare- 
sis ") is CLINICALLY a combination of the 
symptoms of TABES DORSALIS and those of 
GENERAL PARESIS. The COURSE of TABO- 
PARESIS is likely to be from a characteristic 
tabes dorsalis (often of years' standing) to a char- 
acteristic general paresis; the ultimate paretic 
picture is likely to retain various characteristics 
of tabes. The LABORATORY TESTS in the 
paretic phase are characteristic of ordinary (non- 
tabetic) general paresis. The PROGNOSIS, 
after the paretic phase has arrived, is apt to be 
that of general paresis. 



Case i6. Joseph Sullivan, a waiter, 50 years of age, sought 
assistance at the Psychopathic Hospital voluntarily. His 
complaint of severe and lancinating pains in the legs, difficulty 
with his gait, and a feeling of constriction about the waist, 
was forthwith suggestive of tabes dorsalis. He was a rather 
poorly nourished, white-haired man, with a drooping of the 
left side of the face. The pupils reacted sluggishly to light, 
the right somewhat better than the left. A marked Romberg 
reaction could be demonstrated. Ataxia In walking was 
marked. There was some incoordination of the hands, con- 
siderable tremor, and writing was poorly performed. The 
ankle- jerks and knee-jerks were absent. On the whole, 
the diagnosis of Tabes Dorsalis was clear enough. 

The most appealing situation was mental. Sullivan was 
exceedingly apprehensive about his condition on the ground 
that it was growing progressively worse; if it was to get 
worse, Sullivan feared he would commit suicide. From his 
own account, he had become irritable, quick-tempered, and 
often unreasonable. As usual In these cases, the question 
arose whether the depression was psychopathic or natural. 

While In the hospital things shortly came to a crisis. In 
the midst of a fit of depression, Sullivan attempted suicide 



SYSTEMATIC DIAGNOSIS 



93 



TABETIC SYMPTOMS AND SIGNS IN ORDER 
OF THEIR FREQUENCY 

ANALYSIS OF 250 CASES 

PER CENT 

1. ROMBERG SIGN 96.4 

2. ABSENT KNEE-JERKS 90.0 

3. LANCINATING PAINS 88.4 

4. STAGGERING GAIT 87.2 

5. ARGYLL-ROBERTSON PUPIL 80.0 

6. ATAXIA IN UPPER EXTREMITIES 68.2 

7. SPHINCTER DISTURBANCES 67.6 

8. SENSORY DISTURBANCES 58.2 

9. VISUAL DISTURBANCES 43.6 

10. PARESTHESIA AND NUMBNESS OF FEET AND 

LOWER EXTREMITIES 42 .8 

11. GIRDLE SENSE 31.2 

12. PTOSIS OF^EYE-LIDS 23.2 

13. PARESTHESIA OR NUMBNESS IN HANDS OR 

UPPER EXTREMITIES 13.6 

14. STRABISMUS 12 .0 

15. VISCERAL CRISES 12.0 

16. LOSS OF SEXUAL DESIRE 11 .5 

17. CHARCOT JOINTS 9.2 

18. VERTIGO 4.0 

19. MAL PERFORANS 3.2 

20. PAIN IN JOINTS 2.8 

21. RECTAL TENESMUS 2.8 

22. MENTAL DEGENERATION (other than paresis) 2.4 

23. HEMIPLEGIA 2.4 

24. VESICAL TENESMUS 2.0 

25. DIFFICULTY IN ARTICULATION 2.0 

26. DEAFNESS 1.2 

27. ANOSMIA 0.8 

Baldwin Lucke. 
Chart 13 



94 SYSTEMATIC DIAGNOSIS 

by beating his head against the wall. Whether this attempt 
could be regarded psychopathic, however, remained in ques- 
tion. Sullivan had been drinking very heavily although he 
had stopped about six weeks before admission, fearing that 
the alcohol was causing a development of symptoms. The 
remedy was almost worse than the disease because he then 
became more nervous, lost his appetite, and had a marked 
insomnia. 

According to the patient's own history, he had had several 
attacks of gonorrhoea and a syphilitic infection at the age of 
19; that is, some 31 years before admission to the hospital. 
However, the first neurological symptoms of which the patient 
was aware came about 27 or 28 years after infection, namely, 
3 or 4 years before admission, when facial paralysis developed. 
At that time, he had suddenly felt a peculiar sensation in the 
throat and became unable to swallow for a time. His voice 
remained hoarse and low for some time, and his face began to 
droop. The lancinating pains and the ataxia also dated 
back several years. 



1. How shall we evaluate the mental symptoms? The prog- 

nosis of tabes dorsalis is relatively good so far as life is 
concerned, and it might even be possible for Sullivan by 
training to remain capable of being a waiter. The 
manual incoordination was not marked, and possibly 
the manual tremor was in part due to alcohol. Accord- 
ingly, the mental symptoms, such as emotional lability 
and memory defect, were in the foreground of attention. 
In point of fact, the laboratory examinations showed 
positive W. R. in the serum and the spinal fluid, which 
latter also contained 60 cells per cmm., positive glob- 
ulin, and an excess of albumin. The Diagnosis made 
WAS THAT OF TABOPARESIS, meaning thereby a tabes 
associated with appropriate symptoms of a mental 
nature. 

2. How shall the term taboparesis be used? Some use the 

term, as we feel erroneously, for instances of general 
paresis which happen to show crural areflexia (absence 
of knee-jerks). We feel that the best usage of the 
term is for instances in which well-defined symptoms of 
i tabes (as well as of paresis) are present, namely, char- 
acteristic ataxia, lightning pains, and the like. If the 



SYSTEMATIC DIAGNOSIS 95 

term is used more loosely, as above mentioned, then 
practically every case of general paresis might perhaps 
be termed taboparesis, since almost every case of paresis 
does show involvement of the cord as well as of the 
cerebrum. Such involvement may lead to hyperre- 
flexia, hyporeflexia, or arefiexia according to the localiza- 
tion of the process. In true taboparesis, in which there 
is a commingling of the features of tabes with those of 
paresis, we should find the posterior roots of the spinal 
cord affected. The spinal lesions of paresis itself are 
more apt to be intraspinal; that is, confined to the 
nervous system within the pial investment. 

3. Bearing in mind that Sullivan was a waiter, what shall 

be said about the infectivity of these cases? It is 
counted as a rule as negative, since there are no open 
spirochete-bearing lesions. The longer the period since 
infection the less, as a rule, is the chance of contagion 
in syphilis ; and as tabes and paresis occur fairly late in 
the disease, the infectiousness at this stage is practically 
negligible. 

4. Of what differential value is the insight shown by Sulli- 

van into the nature of his symptoms? Kraepelin 
remarks that a genuine insight into the nature of the 
disease does not as a rule occur in paresis. At the 
beginning of the disease, there may sometimes be a 
correct understanding of the nature of the disease and 
of its probable outcome ; but the presence or absence of 
insight into the fact of mental disease is by no means a 
differential sign of practical value. 

5. What is to be said of the occurrence of depression and 

excited states in paretic neurosyphilis? A variety of 
classifications of sub-forms of paretic neurosyphilis 
have been propounded. Kraepelin, for example, deals 
with four: the demented, depressive, expansive, and 
agitated forms, but remarks that the division is merely 
convenient for exposition. The institutional intake 
does not accurately represent the distribution of cases. 
Under psychopathic hospital conditions with the rela- 
tively easy resort to such institutions, the number of 
quiet cases increases; under the less advanced condi- 
tions in Heidelberg, Kraepelin took in 53% demented 
paretics as against 56% at Munich (73% women) under 
the easier conditions of admission. The admissions of 
demented paretics varied from 37 to 56%. The varia- 
tions depend much upon the facility with which the 
cases can be brought to institutions. Where admission 



96 SYSTEMATIC DIAGNOSIS 

is beset with various legal restrictions, the quiet and 
demented cases are more apt to be treated for long 
periods at home. The depressive type of paretic 
neurosyphilis forms a much smaller group, according 
to Kraepelin, as only about 12% of his Heidelberg ad- 
missions were of this type, and still fewer of his Munich 
admissions. Other authors give percentages as high as 
16 and 19. The so-called expansive group is larger, 
Kraepelin finding 30% of his Heidelberg cases to be of 
this group, and 21 to 22% of his Munich cases. The 
rarest sub-form of paretic neurosyphilis is the agitated 
form: 6% of Kraepelin's Heidelberg admissions; 14% 
among males and 5% among females in his Munich 
admissions, where the diagnosis of agitated paresis was 
entered on somewhat broader lines. French authors 
(Serieux and Ducaste) have enlarged the number of 
sub-forms of paretic neurosyphilis as follows : Expansive 
27%; sensory 24%; demented 24%; persecutory 3% ; 
depressive 2%; circular 7%; hypochondriacal 7%; and 
maniacal 6%. 



SYSTEMATIC DIAGNOSIS 97 



DIFFUSE (meningovasculoparenchymatous) NEU- 
ROSYPHILIS may look precisely like PARETIC 
NEUROSYPHILIS (" general paresis ") at certain 
periods of clinical and laboratory examination. 



Case 17. The police found Gregorian Petrofski crouching 
on his knees on a Boston sidewalk, attempting to take 
pickets off a fence. Petrofski knew little English; he said 
that he had slept in Poland the night before. He did not 
appear to be alcoholic. 

When he was examined, through an interpreter, he told 
how he had been in America two days, and in Boston two 
years; that he was at the present time in Poland, and that 
his brother had brought him to the hospital and left him 
there. 

The physical examination showed Petrofski to be well 
developed and nourished. His pupils were somewhat dilated 
and reacted somewhat slowly to light and accommodation. 
Neurologically, there was nothing else abnormal found upon 
systematic examination although, through lack of cooperation, 
sensory and coordination tests proved difficult if not impos- 
sible. There was a large ulcer on the under surface of the 
glans penis, with several small smooth scars on the upper 
surface. There was a purulent discharge from the external 
meatus. There were exostoses of both tibiae. 

The initial diagnosis had to consider uremia and diabetes, 
which could be easily excluded on examination. Alcoholism 
was excluded through absence of alcohol on the breath. 
There remained such diagnoses as epilepsy, some post-trau- 
matic condition, or meningitis, to say nothing of the hypoth- 
esis of syphilis raised by the tibial exostoses and the lesions 
of the penis. The hypothesis of trauma was given up, as 
well as epilepsy and meningitis upon the data of the lumbar 
puncture. The spinal fluid proved to be clear but with 
enormous amounts of globulin and albumin, 80 cells per cmm., 
a " paretic " gold sol reaction, and a positive spinal fluid 



98 SYSTEMATIC DIAGNOSIS 

W. R. (the serum W. R. was also positive). Accordingly, it 
was clear that the case was one of neurosyphilis. 

Treatment was instituted with injections of mercury sal- 
icylate, a grain and a half twice a week, and potassium iodid. 
After some weeks, diarrhoea and salivation with marked 
symptoms of mercury poisoning set in; the treatment was 
suspended, but later re-instituted. In a few weeks Petrofski 
was apparently quite well, the spinal fluid tests had all become 
negative, as had the serum W. R. 

Petrofski now began to pick up a good deal of English, and 
gave a consistent narrative of his past life, although the 
period just prior to and during his early stay In the hospital 
has remained blank. Without further treatment Petrofski 
has remained well for over a year. 

I . Does the ' ' paretic ' ' gold sol reaction mean general paresis ? 
In connection with this general question, a brief sum- 
mary of the significance of the gold sol reaction in this 
group may be made, (i) Fluids from cases of general 
paresis In the vast majority of cases will give a strong 
and fairly characteristic reaction, especially if more 
than one sample Is tested. (2) Very rarely general 
paresis fluid will give a reaction weaker than the char- 
acteristic one. (3) Fluids from cases of syphilitic 
involvement of the central nervous system other than 
general paresis often give a weaker reaction than the 
paretic, but in a fairly high percentage of cases give 
the same reaction as the paretics. (4) Non-syphilltic 
cases may give the same reaction as the paretics ; these 
cases are usually chronic inflammatory conditions of 
the central nervous system. (5) When a syphilitic 
fluid does not give the strong " paretic reaction " it is 
presumptive evidence that the case is not general paresis, 
and this test offers a very valuable differential diagnostic 
aid between general paresis, tabes, and cerebrospinal 
syphilis. (6) The term " syphilitic zone " is a mis- 
nomer, as non-syphilitic as well as syphilitic cases give 
reactions in this zone, but no fluid of a case with syphi- 
litic central nervous system disease has given a reaction 
out of this zone, so that the finding may be used nega- 
tively; and any fluid giving a reaction outside of this 
zone may be considered non-syphilitic. (7) Mild re- 
actions may occur without any evident significance, 



SYSTEMATIC DIAGNOSIS 



99 



FREQUENT SYMPTOMS IN DIFFUSE AND 
VASCULAR NEUROSYPHILIS 

(" CEREBRAL » AND " CEREBROSPINAL SYPHILIS ") 

PUPILLARY DISORDER 

HEADACHE 

VERTIGO 

INSOMNIA 

DROWSINESS 

CHANGE IN DISPOSITION _ 

Irritability Slow thinking 

SEIZURES 

PARALYSES 

Permanent Transient 

APHASIA 

HEMIANOPSIA 

SENSORY DISTURBANCES 

GASTRIC CRISES 

SPHINCTER DISTURBANCES 

INTRACRANIAL PRESSURE SYMPTOMS 

POLYURIA, POLYDYPSIA, GLYCOSURIA 

MENIERE'S SYNDROME 

NYSTAGMUS 



Chart 14 



lOO SYSTEMATIC DIAGNOSIS 

while a reaction of no greater strength may mean marked 
inflammatory reaction. (8) Tuberculous meningitis, 
brain tumor, and purulent meningitis fluids character- 
istically, though not invariably, give reactions in higher 
dilutions than syphilitic fluids. (9) The unsupple- 
mented gold sol test is insufficient evidence on which to 
make any diagnosis, but used in conjunction with the 
W. R., chemical and cytological examinations, it offers 
much information, aiding in the differential diagnosis 
of general paresis, cerebrospinal syphilis, tabes dorsalis, 
brain tumor, tuberculous meningitis, and purulent men- 
ingitis. (10) We believe that no cerebrospinal fluid 
examination is complete for clinical purposes without 
the gold sol test. 

See Appendix B for technical details. 
2. What is the relation of the tibial exostosis to neuro- 
syphilis? The syphilographers have always stressed 
the tibial lesions in the diagnosis of syphilis. Although 
not so much attention has been paid to these and 
kindred osseous lesions in neurosyphilis, yet we have 
frequently found such lesions and they afford an im- 
portant auxiliary means of diagnosis. 



SYSTEMATIC DIAGNOSIS lOI 



A POSITIVE SERUM Wassermann reaction with 
a NEGATIVE FLUID Wassermann Reaction may 
be found in NEUROSYPHILIS, particularly in 
VASCULAR NEUROSYPHILIS: the remaining 
signs in the fluid, although frequently positive, may 
even be negative. 



Case 1 8. Frederick Wescott was a promoter, an elderly 
looking man of 60 years. His health had been falling for 18 
months. There had been shortness of breath, dizziness, a 
tired feeling, inability to " get the words he wanted," and 
forgetfulness of names. About eight weeks before examina- 
tion, Wescott had had a convulsion, following which he had 
been unable to express himself at all well. This convulsion 
was not accompanied by loss of consciousness. Besides a 
marked motor aphasia, there was agraphia. 

Physically, Wescott showed arteriosclerosis and a blood 
pressure of 135 systolic, but, except very lively knee-jerks, 
no other reflex disorders or anomalies were discovered. In 
particular, the pupils reacted fairly well. 

There was, perhaps, no special reason to Implicate syphilis 
in the case, yet Wescott gave a history of syphilis at 35 years. 
The W. R. of the blood serum proved positive; that of the 
spinal fluid was negative, and the albumin was but slightly 
increased; there was a very slight amount of globulin, and 
there were 16 cells per cmm. in the fluid. The gold sol re- 
action suggested syphilis. 

We felt entitled to make a diagnosis of Syphilitic Cere- 
bral Arteriosclerosis, regarding the convulsion or seizure 
eight weeks before as due to a vascular insult. The labora- 
tory picture In the spinal fluid in Wescott's case seems to be 
rather characteristic of this group of syphilitic arteriosclerotics. 

I. What is the reason for the negative spinal fluid W. R.? 
The theory would be that the syphilitic lesion is local- 
ized in the vascular system and that the parenchyma 



102 SYSTEMATIC DIAGNOSIS 

is only secondarily, if at all, involved. The W. R. 
producing bodies are accordingly not found in the fluid. 

2. How frequently are several of the spinal fluid tests neg- 

ative, while others are positive? Whereas, clinically 
speaking, the five tests in the spinal fluid (W. R., globulin 
reaction, excess albumin, pleocytosis, and gold sol 
reaction) are each indicative of a pathological con- 
dition in the central nervous system, yet a specially 
intensive study of the distribution of these tests has 
shown that they are prone to occur independently. 
Consequently, we must concede that they do not all 
represent the same inflammatory products and chemical 
conditions. The W. R. producing bodies, the gold sol 
reaction producing bodies, as well as the globulins and 
albumins, have been proved to be separate. Special 
work has also shown that these tests disappear under 
treatment at different rates. There is, unfortunately, no 
doubt that the rate and intensity, presence or absence, 
and the order of disappearance of these tests in either 
treated or untreated cases, do not at all parallel the 
clinical conditions of the patients. 

3. What is the prognosis in vascular neurosyphilis, such as 

in the case of Wescott? The prognosis is identical 
with that of cerebral arteriosclerosis in general, that 
is to say, bad, but with frequent periods of improve- 
ment. In the neurosyphilitic type of arterial disease 
thromboid formation is frequent. Where the lesion is 
chiefly pervascular infiltration, rather than disintegra- 
tion of the vessel wall, improvement may very well 
occur as a result of treatment. Wescott showed slight 
improvement under treatment. He has already lived 
two years since his first convulsion, and three and a 
half years since the onset of symptoms 



SYSTEMATIC DIAGNOSIS I03 



DIFFUSE NEUROSYPHILIS (so-caUed "cere- 
brospinal syphilis ") is often marked by SEIZURES. 



Case 19. Agnes O'Neil, an unmarried woman of 28 years, 
was first examined five weeks after the initial symptoms. It 
appears that she had had certain seizures, with unconscious- 
ness and twitching of the limbs (otherwise not well described), 
followed by confusion of mind and sometimes by a weakness 
of the left side and a difficulty in speaking. Headache had 
been almost constant, as well as pains in the arms and legs. 

Physically, both in general and neurologically, there were 
no signs or symptoms ; mentally, we could discover no symp- 
toms. Syphilis was denied, although possible exposure to 
syphilis was admitted. 

The diagnosis of some form of organic brain disease was 
clear with the picture of convulsions followed by slight aphasia 
with headaches and limb pains. With onset at 28, the most 
frequent cause for such epileptiform seizures is certainly 
syphilis. Examination of the blood and spinal fluid showed 
a positive W. R., in both. The albumin was also somewhat 
increased. The clinical picture suggested a fairly generalized 
meningitic involvement. 

The prognosis in such cases of generalized meningitic 
involvement is in general good, and this principle was illus- 
trated in the O'Neil case, in which the symptoms soon dis- 
appeared under intensive antisyphilitic treatment. In fact 
the spinal fluid W. R. became negative in the course of four 
weeks. The blood serum W. R., however, has remained 
positive despite eight months of active treatment. 

I. Are certain cases of syphilitic epilepsy really cases of 
Jacksonian epilepsy? As a matter of nomenclature, 
Jacksonian cortical epilepsy is usually the result of a 
focal and circumscribed irritative lesion in the cortex. 
Gumma, local syphilitic meningitis, and syphilitic 
vascular lesions, as well as scars consequent upon the 
latter, are among the causes of Jacksonian epilepsy, 



104 SYSTEMATIC DIAGNOSIS 



CONDITIONS IN WHICH CONVULSIONS OCCUR 

NEUROSYPHILIS 

HYSTERIA 

EPILEPSY MAJOR (Grand Mal) 

EPILEPSY MINOR (Petit Mal) 

DEMENTIA PRAECOX 

TOXIC CONDITIONS: 

Asphyxia, Uremia, Alcohol, Absinthe, Lead, Mercury, etc. 

ORGANIC BRAIN LESIONS 

Apoplexy, Meningitis, Intracranial Growths 

STOKES-ADAMS DISEASE 

MALINGERING 

DISSEMINATED SCLEROSIS 



Chart 15 



SYSTEMATIC DIAGNOSIS 105 

along with such other focal lesions as trauma, tumor 
abscess, tubercle, and the like. Even non-syphilitic 
Jacksonian epilepsy has been observed from time to 
time in cases of diffuse intracranial pressure. Jack- 
sonian attacks also have been found in so-called genuine 
epilepsy. Accordingly, we must not conclude from the 
occurrence of Jacksonian convulsions, even though in a 
proved syphilitic case, that the convulsions in ques- 
tion are surely due to a focal lesion, for they may be 
due to diffuse syphilitic lesions. 

2. What is the significance of aphasia in Agnes O'Neil? 
Aphasia is not a characteristic symptom in ordinary 
Jacksonian epilepsy, but the aphasia is another sign of 
focal lesion and forms an added argument against the 
diagnosis of genuine or idiopathic epilepsy. See also 
discussion of aphasia in paretic neurosyphilis under 
CaseLevenson (22). 

-^ What is the behavior of the serum W. R. and the spinal 
fluid W. R. under systematic treatment? Sometimes, 
as in this case, the serum W. R. remains positive and the 
fluid W. R. becomes negative; but in other equally 
well-defined cases, the reverse holds true, and the serum 
W. R. reaction becomes negative whereas the spinal 
fluid reaction remains positive. The obvious conclu- 
sion is that we cannot always be sure even by faithful 
tests of either the serum or the fluid alone, whether the 
treatment has succeeded in abolishing the laboratory 
signs. 

4. Can this case be regarded as one of cure? Not by the 
definition adopted in this book or by the syphilographers 
who take into account not only the nervous system but 
the body which contains it. To be sure, the spinal 
fluid of Agnes O'Neil is now entirely negative and she is 
clinically free from symptoms; yet from the broad 
standpoint of syphilis therapy in general, this patient is 
not cured, as is evidenced by the positive serum W. R. 



I06 SYSTEMATIC DIAGNOSIS 



PARETIC NEUROSYPHILIS (" general 
paresis ") is often marked by SEIZURES. 



Case 20. Lester Crane, a plumber, 37 years of age, came 
to the hospital with a slow and defective speech. Moreover, 
there seemed to be some mental disorder since his answers to 
questions were not always relevant. It appeared that he 
was seeing bugs on the wall. 

Physically, Crane was a well-developed and nourished man, 
with overactive knee-jerks and a Babinski reaction on the 
left side. 

It developed that there was an impairment in hearing. 
The pupils reacted well both to light and to distance. The 
patient was very restless and smiled in a silly fashion. His 
memory was decidedly defective in all spheres, and he was 
very slow in the intake of ideas. 

The plumber's wife said that, at about the age of 23 or 24, 
he had a spell of confusion lasting two or three days, with 
peculiar conduct, unintelligible talk, and a good deal of 
weeping. The medical diagnosis at that time took into 
account the fact that Crane was a plumber and was " lead 
encephalopathy." 

However, according to his wife, Crane had acquired 
chancre at about 26 years, was treated mercurially for about 
three years and declared well. He had remained well up to 
about 18 months before entrance, when, without previous 
warning, the patient had a convulsion with the continuous 
movements for about half an hour. He was semi-conscious 
for about 18 hours and vomited continuously. There was 
amnesia for the whole affair on regaining consciousness. In 
a week's time, Crane was entirely well. But six weeks later 
there was another convulsion. Upon removal to a hospital, 
the diagnosis of general paresis was made, and the patient 
was given the Swift-Ellis intraspinous treatment. This 
seemed to be very successful, and the patient discontinued 
treatment after 14 weeks (during which time there had 



SYSTEMATIC DIAGNOSIS I07 

been seven treatments) on the ground that he was entirely 
well. 

However, after discontinuing treatment, there was another 
convulsion in about a month, and further convulsions occurred 
once a month. For six months, however, the patient took 
no treatment, but finally returned to the hospital and was 
given mercury. This treatment appeared to suspend con- 
vulsions again for three months, but at the expiration of 
six months, the patient had three convulsions in one day, 
and several more during the following days. After the last 
of these convulsions, there had been numbness on the right 
side of the body and considerable headache. 

The diagnosis of Paretic Neurosyphilis ("general par- 
esis") is borne out by the laboratory tests. The W. R. of 
the blood serum was, to be sure, negative, but the W. R. of 
the spinal fluid was positive, and there was a " paretic " type 
of gold reaction, together with other laboratory signs. 

The case well demonstrates that group of paretic cases in 
which convulsions periodically occur, leaving the patient worse 
after each convulsion. Treatment with salvarsan was insti- 
tuted, and mercury and iodid was given by mouth. During 
the period of eight months which have now elapsed since the 
beginning of this treatment, there have been no convulsions; 
there has been a great improvement in the memory, the hear- 
ing has improved, the W. R. in the spinal fluid is much less 
intense, the gold sol test has become negative, and the other 
tests are all less intense. 

The patient, however, has not been entirely well, for in 
place of the generalized convulsions, he has had minor sei- 
zures, beginning as a rule with a tingling sensation in the 
right hand, extending up the arm, down the trunk and leg, and 
through the right side of the face, with a bitter sensation on 
the right half of the tongue. The patient maintains that 
this sensation is absolutely confined to the right half of the 
body (in this connection we may recall case Morton (i), in 
which there was also a hemiplegia together with other ap- 
parently hysterical symptoms at several times during the 
long course of a disease with abundant structural correla- 
tions). During these minor seizures, the patient is unable 



I08 SYSTEMATIC DIAGNOSIS 



LOSS OF DEEP REFLEXES 

NEUROSYPHILIS 

NEURITIS 

(alcohol, diabetes, diphtheria, lead, arsenic, tubercle, cachexia, etc.) 
Peripheral nerves sensory or motor 

PERIPHERAL NERVE PALSIES 

TEMPORARILY FROM COMPRESSION BY TURNIQUET 

FRIEDREICH'S ATAXIA 

SUBACUTE COMBINED DEGENERATION OF POSTERIOR 
AND LATERAL COLUMNS 
Posterior column disease ■ 

FOCAL LESION IN GRAY MATTER OF CORD 

INFANTILE PARALYSIS (ACUTE ANTERIOR POLIOMYELITIS) 

PROGRESSIVE MUSCULAR ATROPHY 
(chronic anterior poliomyelitis) 
Anterior cornua of cord 

AMYOTROPHIC LATERAL SCLEROSIS 

SYRINGOMYELIA 

THROMBOSIS OF ANTERIOR SPINAL ARTERY 

LANDRY'S PARALYSIS 

Anterior cornua and peripheral motor nerves 

MYOPATHIES 

(pseudohypertrophic and atrophic types) 
Muscle itself 

AMYOTONIA CONGENITA 

FAMILY PERIODIC PARALYSIS 

(during attacks) 

INCREASED INTRACRANIAL PRESSURE 

(especially hydrocephalus and tumors of posterior fossa) 

PNEUMONIA 

IMMEDIATELY AFTER ATTACK OF MAJOR EPILEPSY 
(post-epileptic coma) 

TOXIC COMA 

(uremia, morphine, etc.) 

DURING SPINAL ANESTHESIA 

COMPLETE TRANSVERSE LESION OF CORD 

PuRVES Stuart 

Chart i6 



SYSTEMATIC DIAGNOSIS IO9 

to talk, although he does not lose consciousness and is en- 
tirely aware of everything going on about him. These 
attacks have of late been growing somewhat less frequent. 

1. What is the cause of the negative serum W. R.? It is 

claimed that 3 to 5% of all cases of general paresis 
yield a negative blood serum. In this particular case, 
there had been considerable treatment, including some 
Swift-Ellis treatment, so that it may be that this 
treatment had reduced a formerly positive blood serum 
W. R. to a negative one. 

2. What is the nature of the typical seizures of general 

paresis? The most frequent seizures are epileptiform 
and bear a general resemblance to cortical epilepsy; 
but more rarely these seizures resemble the ordinary 
epileptic attack or consist of a violent general shaking 
of the whole body. A variety of initial minor disorders 
usher in the attacks : the temperature is often increased. 
The attacks are over after one or at most after a few 
hours. Kraepelin speaks of one that lasted 14 days. 
Sometimes a status paralyticus develops, suggestive of 
the status epilepticus. Another rarer form of charac- 
teristic seizure is the apoplectiform, which can hardly 
be told from an ordinary stroke, and may be followed 
by the usual post-apoplectic phenomena. A good 
many of the strokes leading to sudden death in middle 
life are probably cases of neurosyphilis although often 
set down as early arteriosclerosis of a non-syphilitic 
nature. Besides the epileptiform and apoplectiform 
seizures, there are certain seizures of a less definite and 
complete nature, ranging from simple fainting spells, 
dizzy spells and petit mal attacks, to various special 
forms of irritative muscular contractions and temporary 
speech disorders. Sometimes these attacks occur with 
complete preservation of consciousness. Transient par- 
esthesias, visual field defects, and especially attacks of 
vomiting, which, according to Kraepelin, may precede 
paresis by years (of course in this connection gastric 
crises of tabes must be thought of) , may be counted as 
sensory seizures. 

3. What is the proportion of paretic cases developing 

seizures? Figures vary from 30 to 90%. According to 
Kraepelin, seizures occurred in 30 to 40% of his cases 
at Heidelberg; he was of the impression that treatment 
in bed had reduced the number of seizures. 65% of 



no SYSTEMATIC DIAGNOSIS 

paretics admitted to Munich (under very free conditions 
of admission) were determined to have shown seizures 
before their admission to the hospital. Seizures are 
said to be somewhat more frequent in men than in 
women. These paretic seizures are not due to either 
hemorrhages or vascular plugging — at least in the 
vast majority of cases — and must be ascribed to the 
effects of microscopic injuries. 

4. What is the effect of seizures upon the future course of 

paretic neurosyphilis? The current idea as expressed, 
for example, by Mercier, is that " immediately after 
each crisis the patient is much worse than he was before 
it, and thereafter there is some improvement, but he 
never improves up to the point at which he was before 
the occurrence of the crisis." That is, " The course of 
the disease is one of sudden plunges, each deeper than 
the last, each followed by a gradual recovery that is less 
complete than the recovery from the previous plunge." 

5. During what period of the disease are seizures most 

common? Late in the disease many cases have convul- 
sions, even though there were none for the first year 
or two. In other cases the convulsion is the first 
indication of paresis. 



SYSTEMATIC DIAGNOSIS III 



DIFFUSE (non-paretic) NEUROSYPHILIS (" cer- 
ebrospinal syphilis ") is often marked by APHASIA. 



Case 21. Martha Bartlett, a woman of 40 years, was 
brought to the Psychopathic Hospital aphasic, or at least 
unable to talk distinctly enough to be understood, or even 
to give name and address. The police had found her wan- 
dering aimlessly about the streets. Although she was well- 
dressed, she was mud-bespattered and apparently had not 
changed her garments for several days. It shortly developed 
that the patient, although unable to express herself either in 
words or by writing, could understand everything that was 
said to her and could indicate by the monosyllables yes or no 
whether she agreed or disagreed with statements made. 
It was thus determined that she was pretty well oriented. 
She was able to understand both speech and printed words. 
Although she approximated more than is at all common a 
pure type of motor aphasia, it appeared that there was a slight 
involvement on the sensory side, especially in the sphere of 
visual imagery. 

Neurologically, the patient showed moderate strabismus, 
slight deviation of the tongue to the right, and considerable 
tremor on protrusion of the tongue. The right side of the 
palate hung lower than the left. The ankle and arm reflexes 
were possibly more active on the left side, and the left grasp 
was somewhat better than the right. Both knee-jerks were 
active, but again the reflex on the left side was more active 
than the right. No other abnormalities of reflex were deter- 
mined. There was no Rombergism but the gait was some- 
what ataxic. For the rest, the physical examination was 
normal. The blood pressure was 120 systolic, 85 diastolic. 

The ready suspicion was that the case was one of apoplexy 
of slight degree with post-apoplectic phenomena. Upon 
investigation, this suspicion was confirmed since it appeared 
that Mrs. B. had been apparently quite well until about six 
months before admission, when without particular warning 



112 SYSTEMATIC DIAGNOSIS 

CONDITIONS IN WHICH SPEECH DEFECT 
IS FOUND 

NEUROSYPHILIS 

HYPOGLOSSAL PARALYSIS 

FACIAL PALSY 

PARALYSIS OF PALATE (Post Diphtheritic) 

BULBAR PALSY" 

PSEUDOBULBAR PALSY 

MYOPATHY— FACIO-SCAPULO-HUMERAL TYPE OF LAN- 
DOUZY AND DEJERINE 

MYASTHENIA GRAVIS 

FRIEDREICH'S ATAXIA 

LARYNGEAL TABES 

ALCOHOLIC INTOXICATION 

POST HEMIPLEGIC 

LENTICULAR DISEASE , 

BILATERAL ATHETOSIS 

MULTIPLE SCLEROSIS 

DEAF MUTISM 

PARALYSIS AGITANS 

CHOREA 

STAMMERING 

TICS 

HYSTERICAL APHONIA 

Chart 17 



SYSTEMATIC DIAGNOSIS II 3 

she began to act strangely and promptly fell into a series of 
convulsions. These convulsions would begin with twitchings 
of the face, and then spread throughout the body. There 
would be a period of unconsciousness for two or three hours. 
It is not certain how many of these convulsive seizures the 
patient had. At all events she is reported to have recovered 
therefrom completely, remaining well for three months ; where- 
upon, suddenly, while visiting a friend, she suffered a paraly- 
sis of the left side of the body. She remained dazed and 
had hospital treatment for about a week. Ever since this 
left-sided paralysis, the aphasic condition above described 
has persisted. 

Such a phenomenon has often been dismissed in the past 
as due to an early arteriosclerosis, but most neurologists and 
internists of today would look beyond the diagnosis of mere 
arteriosclerosis and consider syphilis. The only suggestive 
feature in the case, aside from the post-apoplectic reflex 
disorder and spastic phenomena, is the irregularity and di- 
minished light reaction of the pupils. Our suspicions were 
confirmed by the positive serum W. R. The W. R. of the 
spinal fluid proved, however, to be negative. There was a 
moderately strong gold sol reaction of the syphilitic type. 
There was a slight excess of albumin, and there was an ex- 
ceedingly slight amount of globulin. There was but one cell 
per cmm. 

On the whole. It would seem best to consider the case of 
Mrs. Bartlett to be one of Cerebral Arteriosclerosis of 
Syphilitic Origin, and a case In which there is no evidence 
of meningitis or meningo-encephalitis. 

I. What is the explanation of the negative spinal fluid 
W. R.? It may be that none of the W. R. producing 
bodies have gone over into the spinal fluid. It has been 
shown by the work of Weston that the W. R. produc- 
ing body is not identical with the bodies responsible for 
the other tests in cerebrospinal syphilis. Moreover, it 
has been clearly shown that these several tests of the 
spinal fluid do not run at all parallel with one another. 
Especially is it true that the chemical tests do not cor- 
respond at all with the degree or nature of the pleocy- 
tosis. On the whole, when involvement of the nervous 



114 SYSTEMATIC DIAGNOSIS 

system is entirely vascular, it is not only theoretically 
proper but also practically common, to find a spinal 
fluid negative to several tests. 

2. Omitting consideration of the syphilitic gold sol of this 

case, what conclusion could be drawn from the albumin 
and globulin findings? It would not be warrantable to 
assume syphilis since it is a common finding after 
cerebral hemorrhage due to non-syphilitic arterioscler- 
osis to find excess albumin and also globulin in the 
spinal fluid. Occasionally, also, pleocytosis occurs in 
cases of cerebral hemorrhage even when the hypothesis 
of an active meningitis can be excluded. We may recall 
in this connection the pleocytosis in so-called menin- 
gitis sympathica of certain brain tumors. (See also 
the case of Milton Safsky (48), a case of brain tumor in 
which there was an excess of albumin, a large quantity 
of globulin, and a pleocytosis of 146 cells per cmm.) 

3. What can be expected from treatment in these cases of 

vascular cerebral syphilis? The condition offers very 
little opportunity for therapeutic results. However, 
antlsyphllitic therapy is indicated to prevent If possi- 
ble further progress of the lesions. Since the lesions are, 
however, vascular, and since It must remain a question 
how far these vascular lesions are due directly to spiro- 
chetal action, and since In any event It may be diffi- 
cult to reach the spirochetes thus active, perhaps it is 
best to place most reliance on potassium iodid. In any 
event, potassium lodid should be given. Salvarsan 
and mercury are also Indicated. It is common to warn 
against administration of large doses of salvarsan in 
this type of case on the ground that further vascular 
ruptures may be produced. (See Friedberg, 108.) 

4. If we conclude that the aphasia of the Bartlett case is 

due to vascular disease, can we conclude a relation 
between this vascular disease and vascular tension? It 
is not safe to draw such a conclusion. The Bartlett case 
itself showed low blood pressure. To be sure, some 
cases of neurosyphilis show high blood pressure from 
which one draws the ci la mode clinical conclusion to the 
effect that the kidneys are probably Involved In the 
arteriosclerosis; but other cases do not show a high 
blood pressure but may in fact show a low blood pres- 
sure. The vascular disease doubtless responsible for 
the aphasia in the Bartlett case is probably not at all 
an effect of blood pressure conditions, but is, on the 
contrary, an effect of local syphilitic vascular lesions. 



SYSTEMATIC DIAGNOSIS II5 



PARETIC NEUROSYPHILIS (" general paresis ") 
is often marked by APHASIA. 



Case 22. Meyer Levenson, a traveling salesman of 36 
years, had for the last two or three years been undergoing a 
change of disposition, quite interfering with his work. He 
had begun to take unreasonable aversions to people, had 
become irritable and emotionally depressed, and often fell to 
weeping without cause. 

About nine months before hospital observation, it seems 
that a trunk-cover had fallen on Levenson' s head, and there 
is some question as to whether he did not have a convulsion 
at that time. However, a month later he had a definite 
seizure, followed by speech disorder, a slight paralysis, and a 
staggering gait. Four weeks later, however, he had gotten 
over these post-convulsive difficulties and had gone back to 
work. 

At his work, he became tired easily, his gait and speech 
did not seem entirely normal, and there was a considerable 
memory disorder. After five more months, another attack 
of a convulsive nature, with twitching of hands and face and 
tongue-biting occurred, and the attending unconsciousness re- 
mained for two days. Again improvement followed, though 
without ability to return to work. Four (?) months later 
there were several severe convulsions and Levenson would 
remain unconscious for a day or two at a time. Restless- 
ness, irritability, and irrational talking followed. 

Physically, the patient was fairly well developed and nour- 
ished; blood pressure 168 systolic, 68 diastolic; pupils re- 
acted very sluggishly to light. There was a marked motor 
aphasia, which the patient recognized as a speech difficulty. 
On the whole, however, Levenson was very euphoric and was 
entirely sure that he was improving and would surely get well. 

Shortly after entrance, Levenson had a severe convulsion, 
with unconsciousness. The movements were mainly on the 
right side of the body, and there was a post-convulsive weak- 



Il6 SYSTEMATIC DIAGNOSIS 

ness of the right side for several days, followed by a slow- 
recovery of strength. 

The course of the disease — convulsions followed by im- 
provement — is very characteristic of a paretic onset. The 
laboratory findings were in all respects confirmatory. It was 
rather striking that a permanent motor aphasia followed the 
convulsions in this case, since the seizures of paresis do not 
in the vast majority of cases leave permanent paralyses. 
The course of the disease continued to show convulsions, 
which would in each instance leave him at a lower terrace of 
capacity than had been before shown. The patient died 
four years after the onset of symptoms of a general asthenia. 
With the exception of the permanent motor aphasia, this 
case might be regarded as a fairly typical one of general 
paresis. 

1. What is the general nature of speech disorder in paretic 

neurosyphilis? Speech disorder is, along with the 
pupillary changes, one of the most important clinical 
symptoms in paretic neurosyphilis. There are aphasic 
and articulatory disturbances. The aphasia that ac- 
companies paretic seizures is of a transient nature as a 
rule. A case with such long-standing motor aphasia 
as shown by Levenson is not common. Paraphasia, with 
incorrect naming of objects, may last longer. The so- 
called " sticking " phenomenon is often observed. 
Word deafness is said to be rarer but is difficult to test 
on account of the patient's dementia. Agrammatism 
(incapacity to form correct sentences) is sometimes 
observed. But the most characteristic disorder is 
in the syllabic composition of words. Syllables are 
left out (" medaltricity " for medical electricity), or 
fused (" exity "), or doubled (" electricicity "). Be- 
sides the central speech disorders of which the above 
are examples, there are disorders in articulation, which 
at first occur as a consequence of paretic seizures or in 
states of excitement, but later become permanent. These 
are divided into paretic and ataxic disturbances. 

2. What is the structural basis of these forms of aphasia? 

It is believed that they are due to microscopic changes, 
not to coarse destructive lesions. 



^'^'^j MASS.] 



[BROOKLINE, 



^j^y^P^V^j^p^ ^^n/v '^Vt- tX-ir *> , [BROOKLINE, MASS.; 

Mss. of Levenson, case 22. Paretic neurosyphilis. Tremor, misspelling. 
Metathesis of letters (Bk, not Br) omission of letters (Book). 

A 






'C%.a»-o'-'<^^ ^t,<f^ -iC-e^ '^^^!»<r 



/'yi^CyVL^ ,/^CJA£y''^^ yf ju,^^ ^^^^ s^v6 the Commonwealth 

/ ^ "^ f of Massachusetts] 

Mss. of Safsky, case 48, brain tumor. Tremor not marked. Misspelling, omission 
of letters. Wrong letters (h in hweth). 



-^^^^ /^5»-«/5E 




Mss. of Halleck, case 31, cervical tabes. No brain disorder. Pen-holding and bearing- 
on difficulties. Crowding of phrases result of ataxia. 

Mss. of Collins, case 61, paretic neurosyphilis. One misspelling (-chusretts) ; not psycho- 
pathic? Characteristic tremor. 



SYSTEMATIC DIAGNOSIS II7 



REMISSIONS of identical appearance occur in 
PARETIC and in DIFFUSE (non-paretic meningo- 
vascular) NEUROSYPHILIS. 



Case 23. Thomas Donovan, a merchant 44 years of age, 
acquired syphilis according to his own story at the age of 31, 
and he was at that time treated at a|Well-known watering- 
place with mercurial injections. Later he continued treat- 
ment under his family physician, and at 34 was pronounced 
cured. However, four years later — that is seven years 
after his initial infection and in his 38th year — he had his 
blood examined and it proved positive. He was accordingly 
treated by salvarsan and his W. R. became negative. The 
story did not end there, however, for at 43, mental symptoms 
appeared of the nature of depression and a diagnosis of paresis 
was made. He was released from the institution against 
advice at that time, and without treatment, made a partial 
recovery. 

A sudden outburst of violence brought Mr. Donovan to 
the Psychopathic Hospital; he was very surly, combative, 
and difficult to manage, standing 6' 2", and weighing 210 
pounds. He was oriented only fairly well and his surliness 
was streaked with humor. He facetiously said that the 
Psychopathic Hospital was the largest hospital in the country, 
and that it was, in fact, a horse hospital; that he had come 
because he liked the surroundings, not to make money; that 
he was the healthiest man in the world, never having been 
sick; that the Psychopathic Hospital was a club, for which 
you have to get somebody to propose your name. There 
was amnesia and no knowledge of current events. He 
regarded the food as poisoned, refused to eat, and was very 
irritable and untidy. 

Physically, there were few abnormalities, but the pupils 
failed to react either to light or accommodation, and the 
knee-jerks and ankle- jerks were absent. There was a slight 
Rombergism. There was a marked speech defect to test 



Il8 SYSTEMATIC DIAGNOSIS 



ATAXIA OR INCOORDINATION 

NEUROSYPHILIS 

LESION OF PERIPHERAL SENSORY NERVES 

DIVISION OF POSTERIOR ROOTS 

TUMORS OR CHRONIC SCLEROSIS OF POSTERIOR COL- 
UMNS 

SUBACUTE COMBINED DEGENERATION 

VESTIBULAR ATAXIA 

FRIEDREICH'S ATAXIA 

FAMILY PROGRESSIVE HYPERTROPHIC NEURITIS 

THROMBOSIS POSTERIOR INFERIOR CEREBELLAR ARTERY 

MARIE'S HEREDITARY CEREBELLAR ATAXIA 

LESIONS OF CEREBELLUM, TUMORS, ETC. 

WRITERS' CRAMP 

PREHEMIPLEGIA 

MULTIPLE SCLEROSIS 

PSEUDO-SCLEROSIS 

HYSTERIA 



Chart i8 



SYSTEMATIC DIAGNOSIS II9 



CONDITIONS IN WHICH VERTIGO IS FOUND 

NEUROSYPHILIS 

HEAD TRAUMA 

CEREBRAL ANEMIA AND HYPEREMIA 

MENOPAUSE 

ARTERIOSCLEROSIS 

RENAL DISEASE 

CEREBRAL HEMORRHAGE AND THROMBOSIS 

INTRACRANIAL TUMORS 

MULTIPLE SCLEROSIS 

EPILEPSY (Aura) 

TOXIC CONDITIONS: 

alcohol, tobacco, constipation 

PSYCHONEUROSIS 

OCULAR DISTURBANCES 

EAR DISEASE 

MENIERE'S DISEASE 

MIGRAINE 



Chart 19 



120 SYSTEMATIC DIAGNOSIS 

phrases. Both serum and spinal fluid W. R.'s were positive; 
the fluid showed 41 cells per cmm., there were large amounts 
of globulin and albumin, and the gold sol reaction was of 
the " paretic " type. 

Salvarsanized serum was injected Intraventricularly through 
a trephine opening in the right frontal region. Injections 
were made through the corpus callosum into the third ven- 
tricle. There was progressive symptomatic improvement 
after each of four injections. In fact, after the fourth injec- 
tion the patient was allowed to leave the hospital despite 
the fact that there was only a slight improvement in the 
spinal fluid findings. The speech defect had entirely dis- 
appeared. (Speech defect, according to many authorities, 
including Kraepelin, is of very grave diagnostic significance.) 
His memory returned. Mr. Donovan is now able to handle 
figures rather extraordinarily well. He now has a good in- 
sight into his delusions and tells stories about them with 
great humor. 

I. What is the definition of a remission in general paresis? 
Remissions form a foil to seizures; just as seizures 
mark a sudden advance in the severity of the disease 
or may even lead to death; so remissions may cause 
a sudden cessation of both mental and nervous phe- 
nomena in the disease. Whereas the seizures occur 
most often, according to Kraepelin, in the demented 
types of paresis, the remissions occur in all cases except 
in the terminal phase. Kraepelin quotes Hoppe as ob- 
serving pronounced remissions of long duration in 
17% of male and 15% of female paretics. Gaupp 
observed marked improvement in less than 10%, and 
very marked improvement indeed in only 1% of his 
cases. Kraepelin states that such improvements are 
most frequent in agitated and especially in expansive 
forms of paresis, and that they are rarer and less com- 
plete in the depressive and demented forms. Sometimes 
the improvement occurs over night, although the full 
extent of the remission becomes complete only gradually, 
perhaps in the course of months. The sensorium clears, 
the disorientation disappears, the delusions retreat, 
and the former delusions are treated as dreams and 
imaginations. There is often a good deal of persistent 
uncertainty as to events during the height of the disease. 



SYSTEMATIC DIAGNOSIS 121 

The nervous disorders are far more obstinate than the 
mental. Still, both speech and writing may often 
greatly improve. 

Cotton in New Jersey found, among 127 cases of 
paresis diagnosticated by modern methods during 
seven years, that remissions occurred in but five, or 
about 4%, lasting from a half to three years. 
2, Does a remission ever amount to a cure? The classical 
case quoted in this connection is one observed by 
Tuczek. This case developed a picture of paresis in 
1876, at the age of 36; and a remission, or cessation, 
of symptoms, occurred in 1878 ; but in 1883, at 43 years, 
the patient developed a tabes without any trace of 
mental disorder, which tabes gradually advanced. By 
the middle of 1898, when the patient was 58, certain 
symptoms of excitement and confusion occurred, which 
led to death with dementia, 22 years after the begin- 
' ning of the disease. Nissl pronounced the cortex to 
be undoubtedly the characteristic cortex of a paretic. 
This observation seems to indicate that a clinical 
remission tantamount to a clinical recovery may occur 
without the death of the spirochetes engaged. This 
observation is to be held in mind in connection with all 
therapeutic work with neurosyphilis. 

Nonne states that during his clinical experience of 
19 years he had followed 10 cases of paresis with ap- 
parent recovery; but of these ten cases, four had to 
be thrown out by Nonne because the apparent recov- 
eries turned out to be only long and almost complete 
remissions, finally issuing in characteristic dementia. 
Of the remaining six cases, perhaps two should hardly 
be counted as paretic and Nonne rather preferred to 
term them cases of syphilitic dementia in the sense of 
a non-paretic cerebral syphilis. At the end, therefore, 
of his review of observations, Nonne found himself 
with four cases of true recovery from paresis. 

Spielmeyer holds that there is no theoretical reason 
why paresis might not be cured, since all the different 
changes that have been described in the disease can 
be halted, and many of them can be repaired. In 
particular, he reminds us that the acute infiltrative proc- 
ess, the neuroglia reaction, and the phagocytic action 
of the large mononuclear cells are distinctly removable 
processes. (See discussion below under Section V, 
for apparent cures and remissions occasionally secured 
under treatment.) 



122 SYSTEMATIC DIAGNOSIS 



REMISSIONS of identical appearance occur in 
PARETIC (" general paresis ") and in DIFFUSE 
(non-paretic) NEUROSYPHILIS. 



Case 24. Michael O'Donnell, a laborer of 48 years, came 
home, one day, at 5 130, complaining of severe headache. His 
wife told him he should lie down and, taking him by the arm, 
tried to help him to the bed. At this moment, O'Donnell 
lost control of both left arm and left leg, and fell, unable to 
move but with consciousness preserved. The wife noted 
that the left side of his face was drawn up and that he drooled. 
He was at once carried to a general hospital, remaining there 
for about three weeks, talking at random in a delirious manner 
and tied in bed. Two intraspinous injections of salvarsan 
were given, and O'Donnell showed considerable improvement 
and went home. 

However, upon his return from the hospital, he became 
very wilful, would not remain in bed, and on one occasion 
actually took the mattress from the bed, carried it to another 
room, and then returned to his own room and slept upon the 
springs. He became irritable and emotional, insisted upon 
going to the hospital, did not go there but upon returning 
home insisted that he had been there. That night, O'Donnell 
left the house only partly dressed. 

It appears that O'Donnell had been excessively alcoholic, 
but that before August 15, when he sustained the left-sided 
hemiplegia above mentioned, there had been no symptoms; 
except that in February he had once been very dizzy. It! 
appears that there had been another dizzy spell, three nights 
before the paralysis, accompanied by a fall and unconscious- 
ness for about 15 minutes. 

O'Donnell was brought to the Psychopathic Hospital some 
six weeks after the paralysis, complaining merely of a slight 
headache and desirous of treatment. There were no mental 
symptoms of any sort. Physically, O'Donnell was in general 
not abnormal (there was a slight pre-systolic murmur and 



SYSTEMATIC DIAGNOSIS 1 23 



TRANSIENT OR FLEETING PARALYSES 

NEUROSYPHILIS 

MYASTHENIA GRAVIS 

MYOTONIA CONGENITA (THOMSEN'S DISEASE) 

PARAMYOTONIA CONGENITA 

MYOTONIA ATROPHICA 

INTERMITTENT CLAUDICATION 

OCCUPATION NEUROSES 

FAMILY PERIODIC PARALYSES 

TETANY 

EPILEPSY MINOR 

HYSTERIA 

MULTIPLE SCLEROSIS 

APOPLEXY 

CEREBRAL THROMBOSIS 



Chart 20 



124 SYSTEMATIC DIAGNOSIS 

a blood pressure of 190 mm. systolic) . The pupils were slight- 
ly irregular, the left larger than the right; both reacted 
sluggishly. Both ears were moderately deaf; the tendon 
reflexes of the left arm and leg were somewhat more lively 
'than those on the right. The systematic neurological ex- 
lamination otherwise revealed no abnormalities. The urine 
was negative. The serum W. R. was positive but the spinal 
fluid reaction was negative. There were but 2 cells per cmm., 
' and there was a very slight trace of albumin. 

1. How shall we account for O'Donnell's transient paralysis? 

We might invoke brain tumor, alcoholic pseudoparesis, 
or some form of neurosyphilis. The diagnosis of brain 
tumor seems quite untenable in view of the absence 
of premonitory symptoms and in the absence of in- 
tracranial pressure. As for alcoholic pseudoparesis it 
is true that the patient was excessively alcoholic. 

However, against these two diagnoses and in favor 
of the diagnosis of Neurosyphilis, are the positive serum 
W. R. and the pupillary reactions (although these are 
short of the true Argyll- Robertson phenomenon). 
Dizziness with retention of consciousness and associated 
with the paralyses mentioned suggests rather a sub- 
cortical than a cortical lesion. We are inclined to 
regard this lesion as probably Thrombotic, and to place 
it possibly in the region of the internal capsule. We 
are inclined to regard the phenomenon as purely vascu- 
lar and as not in this case associated with an encepha- 
litis. We are, however, not entirely satisfied with the 
diagnosis. 

2. What shall be said as to treatment? A full-blown left- 

sided hemiplegia may be produced even when the 
thrombotic lesion is itself exceedingly small. It is 
common to explain this on the basis that there is an 
area of collateral edema about the small necrotic, 
thrombotic, or hemorrhagic area responsible for the 
lesion. In short, numerous neurones are functionally 
rather than structurally affected, or at all events capable 
of early restitution of function. 

3. What is the prognosis in such cases? It appears that 

now and again patients run for several years without 
further trouble, both with and without treatment. 
We are inclined, however, to advocate treatment rather 
than absence of treatment for a variety of reasons. 



SYSTEMATIC DIAGNOSIS I25 

In the first place, vascular lesions may at any time 
become associated with meningitic lesions, and treat- 
ment by salvarsan may perhaps be counted on to head 
off this process; secondly, the treatment with iodids 
may possibly aid in the resolution of a local thrombotic 
process. 

4. What are the prodromal symptoms of cerebrospinal syph- 

ilis? According to Nonne, headache, dizziness, sleep- 
lessness, mental symptoms of the irritability group, loss 
of capacity as to mental work, whether severe or not, 
and loss of capacity for difficult thinking ; also impair- 
ment of memory. Nonne does not regard these phe- 
nomena as characteristic of syphilitic vascular disease, 
and calls attention to the fact that in every organic 
disease the same subjective symptoms occur. The 
triad — headache, dizziness, and impairment of mem- 
ory — is for example now counted as a prodromal symp- 
tom complex for arteriosclerotic apoplexy (Cramer). 
Of course, apoplectic attacks occur without such pre- 
liminary symptoms: particularly, according to Nonne, 
the nocturnal attacks. 

5. Can the fleeting paralysis be of service in differentiating 

the diffuse from the paretic form of neurosyphilis? 
Probably not. In both forms transient paralyses occur 
as well as the permanent ones. In general, however, 
the transient paralyses are more frequent in paretic 
neurosyphilis, whereas the permanent ones occur more 
often in diffuse neurosyphilis. 



126 SYSTEMATIC DIAGNOSIS 



There are cases of NEUROSYPHILIS in which 
the laboratory signs are positive but in which there 
are no clinical signs or sjnmptoms (PARESIS SINE 
PARESI?). 



Case 25. Richard Lawlor* was admitted to the Psycho- 
pathic Hospital, October 29, 1914, being sent there from a 
general hospital where he had gone on account of a self- 
inflicted wound of the wrist, apparently made in a period of 
depression with suicidal intent. Routine notes follow. 

Family History. Paternal grandparents both died of 
heart disease. Maternal grandfather died at seventy-two of 
dropsy. Moderately alcoholic. Maternal grandmother died 
of shock at fifty-six. Father died at age of forty, after an 
illness of eight years, from heart disease. Father all his life 
was subject to fainting spells and headaches. The only pa- 
ternal cousin died at thirteen months of brain fever. Mother, 
aged forty-seven, is, to say the least, eccentric. Says " she 
has several times been given up from tuberculosis." Two 
maternal uncles died of tuberculosis, one from rupture, one 
from heart disease. One uncle who " doesn't know anything 
after he has a teaspoonful of liquor." Several other uncles 
and aunts whose history is not obtained. Patient is mother's 
only child. Mother was twice married. There were sev- 
eral miscarriages by both husbands; patient child by first 
marriage. 

Past History. Patient born thirty-two years ago, full 
term, normal delivery and development. Measles, mumps, 
and chickenpox in childhood. Subject to headaches since 
seven or eight years old. Kicked in the face by horse at 
seventeen or eighteen, not considered serious. Hit by a 
baseball three or four years ago, leaving him hard of hearing 



* Reprinted from an article by Southard & Solomon : 
" Latent neurosyphilis and the question of Paresis sine 
paresi." Boston Medical & Surgical Journal, XXIV, i. 



SYSTEMATIC DIAGNOSIS 127 

on left side. Married ten years ago; no children because 
he says his wife needed an operation. He denies venereal 
disease by name and symptoms. For past ten years has 
had attacks of depression lasting but a short time, but quite 
severe. Never caused him to quit work as a barber and he 
felt better when working. His married life he says was 
fairly happy except for his wife's extravagances, and on this 
account he left her a little over a year ago, and she has 
applied for a divorce, which he is willing that she should 
have, but does not wish to give her alimony. He admits 
moderate alcoholism. 

Present Trouble. Patient states that since he left his wife 
a year ago he has felt sorry a number of times. He has 
wished he had her back. He has felt lonely. He has had 
six or eight periods of depression in that time similar to those 
he has had for many years, lasting two or three days, and 
sometimes a week. These were always precipitated by some 
cause for worry. In these attacks he feels nervous, sleeps 
poorly, has little or no appetite, sweats during his work and 
everything looks black. Several times in these attacks he 
has had suicidal ideas. Ten months ago he considered taking 
corrosive sublimate. For a little over a week before entrance 
to hospital he had been out of work and had been " sporting." 
The day before entrance he had a telephone message from 
his lawyer which upset him somewhat and he walked the 
floor all night. He had just been shaving when the idea of 
suicide came to him. He sat down a minute when suddenly 
the thought " to hell with the world " came to him; he took 
the razor and slashed his wrist. He does not remember draw- 
ing the razor across his wrist. As soon as he saw the blood 
he felt sorry, called his mother, and was taken to an emerg- 
ency hospital and then sent to the Psychopathic Hospital. 

Physical Examination. Patient is a well-developed and 
nourished man thirty-two years of age. Head is normal as 
to size and shape; there are no scars or marks of injury. 
Hair and skin not remarkable in any way. Ears negative 
to external examination. Teeth well kept; two missing, sev- 
eral gold fillings. Tongue very slightly coated. Throat 
negative. Tonsils easily visible without evidence of inflam- 



128 SYSTEMATIC DIAGNOSIS 

mation or exudation. Neck, no thyroid enlargement, no 
abnormal pulsations, no adenopathy. Chest, symmetrical, 
expansion good, resonant throughout. Breath sounds trans- 
mitted normally. No r^les or rubs heard. Heart, no en- 
largement or cardiac dulness. Sounds of good quality, no 
murmurs heard. Rate regular. Pulses equal, regular and 
synchronous, and of good volume and tension. Systolic 
blood pressure 130, diastolic 65. Abdomen, fiat, soft and 
tympanitic throughout; no masses; no tenderness. Liver 
edge not felt, below costal margin. Spleen not palpable. 
Extremities negative, except for incised wound on left wrist. 

Neuromuscular Examination. Pupils are large, round, reg- 
ular, equal and react readily to light and accommodation. 
No nystagmus, strabismus or ptosis. No weaknesses or 
paresis of facial muscles. The tongue projects medially and 
shows no tremor. The triceps and biceps reflexes are readily 
elicited, and are quite active, as are the knee-jerks and ankle- 
jerks. On one occasion it was thought that the tendon 
reflexes were slightly more active on the left than on the right. 
This was never confirmed; always afterwards found equal. 
There was no tremor of extended hands. Abdominal re- 
flexes not elicited. Cremasteric present on both sides. The 
plantar response is flexor. There is no Babinski, Gordon 
or Oppenheim. No Romberg. Coordination tests well per- 
formed, i No speech defect. No sensory disturbances. Urine 
examination negative. 

Wassermann reaction in the serum: Positive, with choles- 
terinized antigen ; negative, with syphilitic fetal liver antigen. 

Wassermann reaction in fluid positive on two occasions. 
Examination of spinal fluid, November 4: globulin ++ + , albu- 
min + + , 100 cells per cubic millimeter; large lymphocytes, 
8 per cent; small lymphocytes, 90 per cent; plasma cells, 
0.7 per cent; endothelial cells, 1.3 per cent. November 11, 
globulin + + + , albumin + + + , cells 18 per cubic millimeter. 
November 26, globulin ++ , albumin ++ , cells 92 per cubic 
millimeter; large lymphocytes, 13.1 per cent; small lympho- 
cytes, 82.1 per cent; plasma, 1.2 per cent; endothelial, 
3. 6 per cent. 

Gold sol, November 4, 5555432100. 

Gold sol, November 26, 3332100000. 



SYSTEMATIC DIAGNOSIS 1 29 

Mental Examination. On entrance to hospital patient 
seemed slightly depressed and a bit irritable. This condition 
lasted two days, after which he was agreeable and apparently 
entirely over his depression. Even during his mild depres- 
sion, however, he talked freely. There was no evidence of 
retardation. He told his story readily. Orientation was 
intact. Memory excellent. Educational knowledge well re- 
tained. There was no evidence of any hallucinations or 
delusions. 

1. Was Richard Lawlor insane? 

There was, then, on the mental and physical ex- 
amination nothing to make a definite suggestion of a 
psychosis, and the most one could think of was a 
psychoneurosis or a cyclothymia of at least ten years' 
duration. The findings in the cerebrospinal fluid and 
the Wassermann reactions, however, give us material 
for thought. Certainly one cannot call the man 
insane; all who saw him agreed on this point. 

2. If Richard Lawlor should some day develop mental 

symptoms, what would be the genesis of the new psycho- 
sis? Though writers such as Fildes and Mcintosh, and 
Swift, have suggested an anaphylactic or hyperallergic 
explanation for the development of symptoms after a 
normal interval ; such a hypothesis could hardly obtain 
in the present case. The hyperallergic hypothesis for 
the development of tertiary neurosyphilis would run 
to the effect that in the secondary stages there had been 
a definite disease of the nervous system, which, however, 
absolutely cleared up, leaving no inflammatory vas- 
cular or parenchymatous relics of Its existence. Noth- 
ing would on this hypothesis remain except a hyper- 
sensltisation of the tissues. In some later period of the 
now clinically normal person, one or more spirochetes 
from a lesion outside the nervous system are carried 
into the nerve tissues and there set up an anaphy- 
lactic or hyperallergic reaction. It is obviously diffi- 
cult to prove the correctness or incorrectness of the 
hyperallergic theory without numerous examinations 
of the spinal fluid, in clinically normal persons after the 
secondaries have passed. The present case, so far from 
demonstrating a normal fluid, demonstrates a highly 
pathological fluid, even though there are absolutely 
no clinical symptoms which could be regarded as of 



130 SYSTEMATIC DIAGNOSIS 

nervous origin. The burden of proof at the present 
time would seem to lie with those who claim hyper- 
allergy in neurosyphilis. We prefer on present evi' 
dence to think that at the conclusion of the secondaries 
a disease process often remains in the nerve tissues 
despite clinical quiescence. 

3. What is the prognosis in the case of Richard Lawlor? 

The prognosis re neurosyphilis is doubtful. We have, 
however, boldly termed the condition PARESIS SINE 
PARESI, meaning thereby to suggest that the patient 
is in considerable danger of the efflorescence of a true 
diffuse or paretic neurosyphilis. We have no means of 
telling, however, whether the positive symptoms would 
be those of a paretic or a non-paretic neurosyphilis. 
As data accumulate regarding these cases of paresis 
sine paresi, we may be able finally to come upon some 
case in which trauma shall bring out the clinical symp- 
toms of neurosyphilis. For discussion of this matter, 
see the case of Bessie Vogel (52) in Part III of this book. 

4. Should Lawlor have been brought to a psychopathic 

hospital? It is a safe working rule to have any person 
who attempts suicide observed. A large percentage of 
suicides occur in psychotic individuals and a suicidal 
attempt is not infrequently the first recognized abnor- 
mality. Immediate observation is a necessary safe- 
guard against another more successful attempt. 



SYSTEMATIC DIAGNOSIS I3I 



Demonstrates SYMPTOMS and LESIONS of 
PARETIC NEUROSYPHILIS ("general par- 
esis "). Autopsy. 



Case 26. John Morrill, 49, an operative in a mill town in 
Essex County, Mass., was described as a " Saturday night 
and Sunday drinker," with a history of very serious long 
sprees at the age of 43. It seems that he had had what was 
called " sciatica " at 35, and was treated in hospital for 
seven weeks at that time. The nature of this sciatica is in 
doubt, but there was a history of syphilitic infection at 36 
years (scar of glans) . 

Morrill had been married twice, and two of the children 
were dead; one daughter was described as " very nervous," 
but there were four children under ten years of age, all re- 
garded as perfectly healthy. 

Morrill had been a mill operative of average capacity, was 
industrious, and had supported his family despite alcoholism. 
The syphilis had been treated with reasonable thoroughness. 

Aside from alcoholism, there had been no symptoms up to 
two months before admission to Danvers Hospital. Then 
there had been insomnia, fatigue, agitation, eruption on foot, 
loss of ten pounds in weight, hypochondriacal fears, appre- 
hensiveness for the future of the children, incoherent talk; 
and just before admission, his talk was described as foolish. 
He had taken to running away and hiding in bushes by a 
pond and in the cellars of other people's houses. 

The patient was of medium height and weight, with thin 
grayish hair and grayish irides; musculature was slender. 
The face was blank in expression, the teeth poorly preserved 
with atrophy of gums, the tongue coated, and the breath 
foul. There was a gummy secretion of the eyelids, an area 
of brownish branny eruption over both clavicles, a number of 
depressed scars over the limbs and back, and another area 
of scaly eruption on the right heel and the sole of the foot. 
The heart area was increased, and the sounds were faint at 



132 SYSTEMATIC DIAGNOSIS 

the base, with the first sound accentuated at the apex. The 
urine showed a trace of albumin. 

Neurologically, the Romberg position was maintained with 
a general tremor and fluttering of the eyelids. In compli- 
cated movements, the patient was slightly ataxic. The 
pupils were irregular, the left being much larger than the 
right. There were no light reactions to be obtained in window 
light. The reaction to accommodation was present, though 
slight. Vision was poor, |-inch capitals could not be read by 
left eye at reading distance. The knee-jerks were diminished 
equally; the Achilles jerks were absent; the other reflexes were 
normal. Upon the sensory side, the patient gave a history 
of pains in the legs at irregular intervals for several years. 
These pains he described as of a darting character. There 
was little or no sensory disorder, although the outer surface 
of the right leg required a deeper pressure to elicit sensation. 
There were no disorders of muscle sense. 

If Morrill was to be trusted, he had been born in Ireland, 
and had come to the United States at the age of 17. He mar- 
ried at 18 ; there had been seven pregnancies by the first wife, 
with one stillborn child ; one child had died at five weeks. 
The four children by the second wife were healthy. The 
first signs of neuritis had occurred at 45 and had received the 
diagnosis neuritis, although no connection between the 
neuritis and the syphilis had been noted. 

The patient entered the hospital July 26, 1 904, and was 
discharged, improved, January 5, 1905. He returned a little 
more than a year later, January 15, 1906, and died March 21, 
1906. The total duration of the disease from the onset of 
mental symptoms may therefore be stated as somewhat under 
two years. When the patient appeared at the hospital the 
second time, he showed a positive Romberg sign, an unsteady 
gait, an ataxia that still was moderate, and somewhat more 
marked tremors, involving fingers, tongue, and face. He 
was now unable to read ^-inch type with the left eye. The 
knee-jerks, formerly diminished, were both exaggerated, the 
left slightly more so. The Achilles reaction, not obtained 
formerly, now appeared on the right side. The pupils reacted 
as before. The sensory loss had become more marked, since 



SYSTEMATIC DIAGNOSIS 1 33 

sharp and dull points could hardly be distinguished. Deep 
pinpricks were not felt in the leg, and heat could not be told 
from cold. 

The speech in 1904 had been somewhat defective (" truly- 
rural " rendered as "tooly lualal," "sifted soft thistles" as 
" thoft thsistles "), and there had been little further develop- 
ment of the speech defect. The handwriting had lost appre- 
ciably in legibility and had become much more tremulous. 
During the first period of hospital observation Morrill had 
what might possibly have been visual hallucinations, but it 
was impossible to tell whether his story of seeing his wife and 
children trying to get in through the window was hallucina- 
tory or a matter of fabrication. Memory was decidedly 
imperfect and few details of recent events could be produced. 
The association of ideas was almost a so-called " flight " 
of apprehensive, fearful ideas, loosely connected, incoherently 
expressed, and dealing chiefly with his work and his children. 
Judgment was imperfect; the height of the room was esti- 
mated as 24 feet, but the height and weight of persons were 
estimated with fair accuracy, and also the length of small 
objects, whose lengths were doubtless remembered rather 
than estimated. The estimate of time elapsing during a 
medical examination was accurate, but the estimate of longer 
durations involving over-night memories was hopelessly 
imperfect. Emotionally, there was a dulling of sensibility, 
an appearance of suspicion and apprehensiveness ; the patient 
fancied himself to be in a hopeless condition as a result of 
syphilis, but at the same time accompanied his statement 
of his hopelessness with laughter. A sample of his hypo- 
chondriacal ideas: " I am all gone; I am good for nothing; 
I am all gone now; I can't drink now; can't write or talk at 
all ; worse than when you saw me first ; nothing in my inside ; 
all wrong through me again; I aint got no swallow now; 
I can't die even; my heart aint much good; I can't hear 
it beat; I don't think it flutters; no life in these hands; 
they are all cold and dead " (pointing to his arms and moving 
them about) . During such a portrayal the patient laughed 
in a silly way. 

During the second hospital stay, Morrill was at first restless, 



134 SYSTEMATIC DIAGNOSIS 

sleepless, profane, imperfectly oriented for time, possibly 
for place, and also for the attendants. A few weeks later he 
became stuporous and confused, and his feebleness and phys- 
ical exhaustion were finally ended by death, March 21, 1906. 
Death was preceded by a semi-comatose condition; a left 
otitis media had developed. 

At the autopsy, it appeared that death was due to an early 
bronchopneumonia associated with acute splenitis and doubt- 
less related to the otitis media of the left side. The body at 
large showed, aside from these acute lesions, a few chronic 
lesions, including slight scars of the left apex, and chronic 
adhesive pleuritis, chronic dififuse nephritis, and aortic and 
coronary syphilis. The aorta showed slight linear and nodular 
markings, with a single small dark ulcer in the upper thoracic 
region, but the aorta did not show the characteristic scarring 
which syphilitic aortas often show. The femoral marrow 
was of a dark red chocolate color. The thyroid appeared 
to be smaller than normal. A slight sacral decubitus had 
developed. 

The description of the head (E.E.S.) is given in full on 
account of the encephalitic lesions shown. These encephalitic 
lesions may be summed up as follows : 

Local cerebral atrophy and sclerosis of the frontal, 
orbital, and central regions, especially of the left oper- 
culum and left supramarginal gyrus. 

Extension of sclerosis to hippocampal gyri with 
effacement of substantia reticularis alba. 

Slight chronic internal hydrocephalus. 

Granular ependymitis (especially of floor of 4th 
ventricle) . 

Compensatory edema of frontal and central pia mater. 

Cerebellar sclerosis (culmen monticuli, lobus culminis, 
lobus cacuminis). 

Spinal sclerosis (grossly evident in the posterior 
columns of the upper thoracic region and of the lumbar 
enlargement) . 
The details are as follows : 

Head : — Bald on top. Hair gray. Scalp normal. 
Calvarium thin, deeply excavated by arachnoidal villi 
to right of vertex. Diploe absent. Dura closely ad- 
herent in bregmatic region. Dura of usual thickness. 



SYSTEMATIC DIAGNOSIS 135 

Sinuses contain cruor clot. Arachnoidal villi slight. 
Pia mater hazy and over sulcal veins porcelain white 
over all of vertex except occipital poles and over flanks 
(notably left). Thickened also around circle of Willis, 
over culmen monticuli and in posterior cerebellar notch. 
Edema of pia corresponding to atrophy of frontal and 
central regions. Cerebral atrophy most marked in 
orbital surfaces of both frontal lobes, in left area of 
Broca, and in left supramarginal region. The ascend- 
ing branch and the ascending ramus of the posterior 
limb of the left Sylvian fossae both readily admit the 
thumb by reason of atrophy of adjacent substance. 
Induration corresponds closely with atrophy, but is 
not more marked about the left Sylvian fossa. There 
is sclerosis of both hippocampal gyri, with loss of the 
substantia reticularis alba. The culmen monticuli and 
lobus culminis are firmer than the clival regions, and 
the lobus cacuminis is again slightly firmer than the 
clival region. Cerebellum a little softer than usual. 
Pia strips with usual readiness from all regions. The 
subplal region of the frontal lobes is a trifle grayer 
than that of the rest of cerebrum. Ventricles slightly 
dilated. Surfaces evenly sanded. Floor of fourth 
ventricle shows numerous coarse, closely set granules. 
Brain wt. 1200 grms. Cord shows a slight increase of 
consistence over one or two upper thoracic segments 
and in lumbar enlargement corresponding with a slight 
graying out of posterior columns. In places there is 
a suggestion of graying out also in lateral columns. 
A few calcified plaques in posterior lumbar pia. 

Analysis of these details shows a number of lesions that 
characterize paretic neurosyphilis (among others, granular 
ependymitis, frontal atrophy, chronic leptomeningitis), but 
the lesions are more than merely frontal, extending as they 
do back as far as the postcentral regions on both sides, and 
even as far as the left supramarginal gyrus. The cerebellar 
involvement although frequent, can hardly be said to be 
characteristic in paretic neurosyphilis. The spinal Involve- 
ment is characteristic of a case which is probably to be re- 
garded as one of taboparesis; that is, of paretic neurosyphilis 
following a number of years after the establishment of tabetic 
neurosyphilis. The aorta Is almost constantly afl^ected by 
sclerosis in paretic neurosyphilis. The absence of diploe in 



136 SYSTEMATIC DIAGNOSIS 

the skull IS not infrequent and the adherent dura mater is 

often found. 

Microscopically, the tissues showed the characteristic 

lesions of Paretic Neurosyphilis; nerve cell destruction, 

fibrillar and cellular gliosis, lymphocytic and plasma cell 

deposits about the small vessels. 

I. What are the clinical evidences of syphilis outside the 
nervous system? The brownish branny eruptions of 
the skin, the depressed scars and the scaly eruption 
on right heel and sole are very suggestive of syphilis. 
Such clinical evidences of syphilis are very important 
in systematic examination. Although the laboratory 
tests are of the utmost assistance in the diagnosis of 
syphilis, the clinical signs should not be neglected, and 
no physician should rest satisfied with laboratory signs 
alone. X-ray diagnosis of bone conditions sometimes 
succeeds when all other methods have failed. 



SYSTEMATIC DIAGNOSIS 1 37 



GUMMA of cerebral cortex verified by operation; 
death. 



Case 27. The presenting picture in the case of David 
Tannenbaum was that of deep dementia, in which condition 
the patient was brought to the hospital. There was a meagre 
history to the effect that about four months before admission, 
he had lost his job in a hotel through lack of further work. 
We heard that at this time he had begun to suffer with ex- 
cruciating pains in the head; at first, worse at night, later, 
worse by day. It appeared that this pain, though it came 
and went, was chiefly localized on the left side of the head. 
For a fortnight, Tannenbaum had been dragging his legs, 
until finally he had become unable to walk at all. 

Pari passu with these developments, Tannenbaum had 
become mentally confused and irritable, and his memory had 
become untrustworthy. For several days before admission, 
an appearance of marked dementia was presented, with slow 
incoherent, or at all events, irrelevant words, and a complete 
disorientation for person. However, his vison had become 
so poor that it would have been hard for him to have rec- 
ognized any one. 

It appeared that the family history w^as entirely negative; 
that the patient was without education but had been physi- 
cally very strong, and had been fairly successful at first in the 
junk business, and later in the clothing business; but latterly 
he had been less fortunate in the clothing business, and 
finally had to resort to work as a laborer around a hotel. 

His wife had had eleven pregnancies with but one mis- 
carriage. Nevertheless, out of the eleven pregnancies, there 
were now but four living children. 

Physically, Tannenbaum was a rather small man; he was 
flabby and looked as if he had recently lost weight. The skin 
showed areas of pigmentation on the face and sides of the 
neck, and some dark copper-colored circular areas, marble- 
size, in the neck (syphilitic?). There was a slight radial 



138 SYSTEMATIC DIAGNOSIS 

arteriosclerosis. The heart was slightly enlarged with distant 
and indistinct sounds. There was a small pedunculated 
growth on the right side of the abdomen. 

The pupils failed to react to flashlight but they reacted to 
sunlight. They both were slightly irregular but were equal in 
size, and reacted in accommodation. There was apparently 
almost complete blindness and extreme deafness. Arm -jerks 
and knee-jerks were absent; there was an occasional slight 
response of the left ankle- jerk, but the right ankle- jerk was 
absent; the left abdominal reflex was very feeble; the right 
absent; the cremasteric reflexes were absent, but there were 
no other abnormalities in the systematic examination. Hand 
grips weak; gait awkward, with right leg held somewhat flac- 
cidly. 

It was significant that percussion over the left frontal and 
parietal regions was able to elicit great pain. Either through 
the patient's deafness or through sensory aphasia, spoken 
language was not understood. The serum W. R. was positive, 
the fluid W. R. negative. 

Diagnosis : The clinical symptoms seem clearly to indicate 
syphilis. The local skull tenderness and impairment of 
vision might well suggest intracranial pressure. Uniting 
these suggestions, we might automatically arrive at a diagnosis 
of cerebral gumma. We have learned to be rather cautious 
of making a diagnosis of gumma of the brain through its 
mere rarity. 

Decompression was suggested and executed. A deep 
growth resembling a Gumma, in the view of the surgeon, was 
discovered. No attempt could be made to remove it. The 
patient died without recovering consciousness. 

I. What is the significance of the negative fluid W. R. in 
this case of cerebral gumma? The W. R. producing 
substances not infrequently fail to appear in the spinal 
fluid from a gumma of the brain. The serum W. R. 
was positive in this case, but even the serum W. R. may 
be negative in cases of gumma, both of the brain and of 
the body at large. It must be remembered that the 
serum W. R. may be negative in paretic neurosyphilis 
(general paresis); the serum W. R. is even more apt 
to be negative in cases of gumma. 




m- 



Gummatous meningitis. Compression of liemisphere. Tissue 
destruction of underlying cortex. 



SYSTEMATIC DIAGNOSIS 139 

2. Is Operative procedure to be advised in cerebral gumma? 

There are cases in which the acute and threatening 
symptoms of heightened intracranial pressure require 
operative treatment simply because the therapeutist 
cannot wait for the effect of antisyphilitic treatment. 
Moreover, antisyphilitic treatment of cerebral gumma 
is not always as successful as that of most syphilitic 
lesions. 

3. Could the Intracranial pressure be caused by other syph- 

ilitic lesions than gumma? A heavy meningitis may 
cause symptoms such as produced by an Intracranial 
tumor. In such a case one will usually find evidences 
of inflammation in the spinal fluid. Cysts caused by 
syphilitic lesions may also produce identical symptoms. 

4. What is the significance of cranial tenderness? Where 

sensitiveness to cranial percussion is not due to a scalp 
lesion it is very suggestive of a tumor underlying this 
point. A gummatous lesion of the cranium Itself, may 
occur without causing pain or increased sensitiveness. 



140 SYSTEMATIC DIAGNOSIS 



CRANIAL NEUROSYPHILIS (focal syphUitic 
extraocular palsy) without mental symptoms. 



Case 28. A chef, Paolo Marini, 28 years of age, reported 
that on awaking one morning, everything appeared double to 
him and that his right eyelid had begun to drop. In the fol- 
lowing month Marini had begun to feel weak and to have 
difficulty in swallowing, as well as at times difficulty in breath- 
ing. The diplopia was found to develop when Marini looked 
to the right. Mentally, the patient was in all respects normal, 
and no other physical signs were found except the diplopia 
and ptosis above mentioned. The W. serum test was positive, 
but the tests of the spinal fluid were negative. 

Diagnosis: " Cerebral syphilis." 

1. What is the anatomical cause of this condition? It is 

thought to be due in a number of cases to a small dif- 
fuse gummatous lesion at the basis cerebri. In the case 
of Marini this lesion appears to have been a little more 
extensive and to have interfered with the tenth and 
twelfth nerves also. 

2. Why is the spinal fluid negative In such a case as that of 

Marini? Head and Fearnsides believe that intra- 
cerebral lues is characterized by a negative spinal fluid, 
under which circumstance one has always to consider 
the possibility of brain tumor or migraine in addition to 
the suspicion of syphilis. 

3. What other causes besides syphilis should one consider 

for the sudden diplopia? Brain tumor, multiple scle- 
rosis, cerebral arteriorsclerosis, tuberculous meningitis, 
trauma and migrainous ophthalmoplegia, are not 
infrequently at the bottom of this condition. Cases 
also occur in which the etiology remains obscure, even 
at autopsy. 
Under anti-syphilitic treatment, Marini slowly improved. 



SYSTEMATIC DIAGNOSIS I4I 



The SIX TESTS in TABETIC NEUROSYPHILIS 
(" tabes dorsalis ") may run milder than in paretic 
neurosyphilis (** general paresis ") and character- 
istically run somewhat like those of diffuse (men- 
ingovascular) neurosyphilis ; in particular, the fluid 
Wassermann Reaction and the gold sol reaction 
are apt to run milder. The clinical course of tabes 
dorsalis is protracted and the prognosis as to life 
is good. 



Case 29. Mario Sanzi, 55 years of age, had been having 
what he called rheumatism since his 43d year. This rheuma- 
tism affected only the hips and legs, had at times been very 
severe, and for two years past had been almost constant. 
Before that time, pains had come at Intervals, lasted a vari- 
able period, and suddenly disappeared. They were of 
knife- thrust character, and could probably be called " lanci- 
nating." In a given attack, these pains would come at 
intervals of seconds or more. There was also a certain 
unsteadiness In locomotion and inability to control the 
vesical sphincter. 

Physically, the patient was entirely normal so far as could 
be made out except neurologically. Argyll- Robertson pupils, 
absence of knee-jerks, and ankle-jerks, Romberg sign, and 
characteristic gait, left no cause for doubting the diagnosis 
of Tabes Dorsalis. The blood and spinal fluid both proved 
positive to the W. R., though the W. R. In the fluid gave a 
negative reaction with o.i cm. and became positive with 
0.3 cm. or more. The globulin was somewhat Increased though 
less markedly so than In paresis. The gold sol reaction was 
" syphilitic " but weak. It Is to be noted that the disease had 
run a 12-years' course before a doctor had been consulted. 
The primary Infection occurred at 32 years, namely, 11 
years before the symptoms began. At the time of his pri- 
mary infection, Sanzl had received several years of treatment, 
chiefly in the form of mercury by mouth. 



142 SYSTEMATIC DIAGNOSIS 

I. What is the value of mercurial treatment of syphilis 
in the prevention of tabetic or other forms of neuro- 
syphilis? '* Fournier strove for many years to con- 
vince the medical profession that a syphilitic patient 
should be treated for at least two years after his in- 
fection, whether the syphilis seemed latent or patent. 
The method of treating only the symptoms he charac- 
terized as the opportunist method; treatment in the 
absence of definite symptoms the preventive method, as 
preventing the later manifestations. That prolonged 
treatment does prevent is shown by Fournier' s figures 
analyzing 2396 cases presenting tertiary signs. These 
he divides into three groups: Group i, comprising 1878 
cases, or 78 per cent of the whole number, having no 
treatment or inadequate treatment — that is mercury 
for less than one year; Group 2, comprising 455 
cases, or 19 per cent, having moderate treatment — 
that is, mercury for one to three years; and Group 3, 
comprising the remaining 19 cases which represent 
only 3 per cent of the whole number, having treatment 
for more than three years." * 

In the light of what we now know concerning latent 
neurosyphilis, it would seem well for patients to be 
followed from time to time with the W. R. on blood 
and spinal fluid after the supposed completion of the 
treatment of primary and secondary syphilis. The 
examination of the spinal fluid is not superfluous, as 
our experience with the so-called paresis sine paresi 
abundantly shows. At the present day it is not good 
practice to assure a patient that he is cured after two 
years of ordinary mercurial treatment without resort 
to frequent spinal fluid tests, even though the serum 
W. R. be negative. 

* Solomon: " How Shall Latent Syphilis be Treated? The 
Prophylaxis of Syphilis of the Central Nervous System." 
Interstate Medical Journal, XXIII, 8. 



SYSTEMATIC DIAGNOSIS 



143 



TABETIC NEUROSYPHILIS (" tabes dorsalis ") 
is often quite ATYPICAL clinically and may even 
show no single symptom warranting the old clinical 
name " locomotor ataxia." 



Case 30. Stephen Green is a case of Tabes Dorsalis with 
active knee-jerks and without locomotor or muscle-sense 
disorder. When observed at the age of 45, it appeared that 
there were but two complaints : lack of control of the vesical 
sphincter and shooting pains in the legs. It appeared that 
the urinary disorder dated back ten years, when there had 
been difficulty in passing the urine. Sounds had been 
passed at the time; occasionally there had been incontinence 
during after years, ascribed by Mr. Green to the passing of 
the sound. However, the physician at that time stated that 
the incontinence was a symptom of tabes dorsalis. The 
incontinence had recently become worse, especially marked 
at night, though also occurring in the day; much worse during 
excitement, and very much worse after taking alcoholic 
drinks. Besides incontinence, there is also difficulty at 
times in passing the urine, as well as dysuria. 

As for the pains in the legs, they had been first noticed 
some three or four years ago and considered to be mild 
rheumatic effects. Now, however, they have grown pro- 
gressively worse and have been the effective cause of giving 
up business. The pains are sharp, darting, pinching, and 
burning, and last, say, about a second with an interval of 
about the same length. The attack will continue sometimes 
for many hours. 

There is a strabismus of the left eye, ascribed by the 
patient to an accident with an umbrella (there had been 
operation without relief). The pupils showed the Argyll- 
Robertson effect and were markedly irregular. Despite the 
divergent strabismus with diplopia, the eye movements were 
well performed although not in parallel axes. Ankle- jerks 
could not be obtained even on reinforcement, but the knee- 



144 SYSTEMATIC DIAGNOSIS 

jerks were lively, and the other deep and skin reflexes proved 
normal. The blood and spinal fluid tests were characteristic 
of tabes dorsalis. 

It appears that the syphilis was acquired by this patient 
15 years before; that is, 5 years before neurological symptoms 
began. Three courses of treatment had been taken at a 
well-known watering-place, and mercury pills had been taken 
for two years by mouth. The patient is married; has no 
children ; there have been no pregnancies. 

1. What causes may be assigned for the absence of children 

in the family of a tabetic? There may be lesions of the 
genital apparatus (orchitis, or more specialized toxic 
lesions). But impotence such as characterized the 
present case must also be taken into account. 

2. What is the therapy for tabetic pains? Pyramidon is 

nowadays much in favor; morphine may be used; 
some authors recommend that the patients be in- 
structed to chloroform or etherize themselves slightly 
for relief of the pain. Surgery of the nerve roots 
may be resorted to in extreme cases. Intraspinous 
therapy, suggested by various authors, seems to exert 
beneficial effect in many cases. 

3. Is the lack of control of the vesical sphincter an unusual 

initial symptom? On the contrary, the more careful 
the clinical observation, according to some observers, 
the more likely is the examiner to find that vesical 
symptoms were the earliest or among the earliest com- 
plaints of the patient. Baldwin Lucke found sphincter 
disturbances to be initial in 8|% of his long Blockley 
series. He found sphincter disturbance to occur in 
some stage of the disease in 67.6%, being exceeded in 
frequency only by staggering gait (87.2%) and lan- 
cinating pain (71.6%). According to Lucke, the most 
frequent initial symptom is lancinating pain in the 
lower extremity, which, it will be noticed, occurred 
also in our case of Stephen Green as an initial symptom 
along with vesical disturbance. Lucke' s figures show 
that paresthesia of the lower extremities (17.6%) and 
weakness of the extremities (16.4%) are the next initial 
symptoms in frequency. 

4. Could the early treatment in the case of Stephen Green 

be considered as adequate? No better answer can be 
given to this question than by quoting from Dr. Joseph 



SYSTEMATIC DIAGNOSIS I45 

Collins,* who probably has done more than any other 
one man in this country in insisting on the need of 
proper treatment of syphilis. As to the adequate treat- 
ment of syphilis he says: 

" It consists in the proper use of salvarsan and mercury 
begun at the earliest possible moment after infection 
and kept up till all biochemical evidence of the disease 
has ceased, while the metabolism of the individual is 
maintained as nearly normal as possible. But the phy- 
sician does not do his whole duty when he has accom- 
plished this. He must solicitously watch the individual 
to see that no evidence reappears for months and even 
years after the apparent cure. As an index of such reap- 
pearance the Wassermann test of the blood serum and 
of the cerebrospinal fluid is the safest guide. 

Until there is a definite unanimity of belief among phy- 
sicians as to when the treatment of syphilis shall be 
begun, and some concert of action as to what consti- 
tutes the adequate treatment of syphilis, we cannot 
hope to make any considerable progress in the preven- 
tion of syphilis of the nervous system, save by educating 
the individual toward infection." 

* Joseph Collins: Syphilis of the Brain, Journal American 
Medical Association, July lo, 1915, Vol. LXV, pp. 139-144, 



146 SYSTEMATIC DIAGNOSIS 



TABETIC NEUROSYPHILIS may produce symp- 
toms chiefly if not entirely in the region supplied 
by the CERVICAL plexus (" cervical tabes ")• 



Case 31. Paul Halleck, 35, was a salesman who had begun 
to find it hard to carry his sample case, since he was unable 
to tell whether or not he had it in his hand. There was not 
only an anesthesia of the hands, but they felt numb and 
there was often a tingling sensation. Of late it had become 
hard for Halleck to dress himself or to write, and these symp- 
toms had been slowly growing worse. There was no other 
complaint. There was, however, a history of a chancre about 
7I years before, which had been followed by a rash and a 
sore throat. There had been treatment with mercury and 
potassium iodid alternating for a period of two years. 

Physically, there was no evidence of disease except neuro- 
logically. The pupils were unequal (the right larger than the 
left) and reacted slowly to accommodation and not at all 
to light. A marked ataxia of the hands was shown in coat- 
buttoning. The finger-to-nose test showed a marked dys- 
metria. Arm- jerks as well as knee- and ankle- jerks were 
absent. There was a slight swaying in the Romberg posi- 
tion but no true Romberg sign. There was no difficulty in 
locomotion. Both blood and spinal fluid proved positive 
to the W. R.; globulin and albumin were increased. The 
gold sol reaction was syphilitic, and there were 85 cells per 
cmm. 

This case is probably not a pure example of Cervical 
Tabes, since the knee-jerks are also absent, and we may 
suppose a degree of lumbar spinal cord changes In addition to 
the cervical changes. It well illustrates, however, that the 
tabetic involvement of the cord may be quite generalized and 
that it may strike high as well as low. 



SYSTEMATIC DIAGNOSIS 1 47 



ERB'S SYPHILITIC SPASTIC PARAPLEGIA. 



Case 32. Margaret Neal, a maid-of-all-work, 36 years 
of age, was committed to a home for inebriates on account 
of her excessive alcohoHsm, but she was shortly transferred 
to the Psychopathic Hospital on account of difficulty with 
locomotion. We found a very marked spasticity in walking, 
with a characteristic scissors gait. The pupils were some- 
what irregular, and although both reacted to light, the 
left reacted far more slowly than the right and the reaction 
failed to hold well. The arm reflexes were very active, and 
the knee-jerks and the ankle- jerks were particularly exag- 
gerated. There was a double Babinski reaction, as well as 
Oppenheim and Gordon reflexes and a bilateral ankle-clonus. 
There seemed to be tenderness over the nerve trunks In the 
back of the leg, below the knee. There was no evidence of 
Incoordination, no Rombergism, no disturbance of sensation, 
no disorder of the special senses, and not even a tremor of 
the tongue or hands. 

Mentally, the patient was entirely negative. 

Diagnosis: Symptomatically, it is entirely clear that 
the patient was suffering from Spastic Paraplegia. One 
would have to consider besides spinal syphilis, also amyo- 
trophic lateral sclerosis, syringomyelia, and spinal cord 
tumor. However, there appeared to be no definite wasting 
of muscles, and the fact that the sensations were intact seems 
to rule out also syringomyelia. There was none of the 
characteristic pain associated with a cord tumor. There was. 
In fact, a strong clinical premonition that the case was one of 
spinal syphilis, simply because syphilis Is the most common 
cause of spastic paraplegia In the adult. The pupillary 
anomalies were also highly suggestive. 

The serum W. R. proved to be weakly positive, as was also 
the gold sol reaction In the zones characteristic of syphilis. 
The spinal fluid examination yielded 14 cells per cmm. There 



148 SYSTEMATIC DIAGNOSIS 

was a positive globulin test and a moderate increase in al- 
bumin. The W. R. of the spinal fluid was negative. 

1. Why was the spinal fluid W. R. negative in this case of 

spinal syphilis? The explanation of negative W. R.'s 
in spinal syphilis is not easy. Possibly, however, in 
the course of years the intensity of the process has been 
reduced and possibly the W. R. has been one of the 
first tests to disappear. 

2. How shall we explain the nerve trunk tenderness? We 

might consider this to be due possibly to an inflamma- 
tion about the posterior roots. On the whole, partly 
on account of the situation of the pains below the knee, 
it seems probable that the nerve trunk tenderness of 
this case is the residuum of an alcoholic neuritis. 

Treatment : Under injections of mercury salicylate, there 
was a rapid improvement. In fact, in the course of several 
months, the patient regained an ability to walk long distances. 
There still remains a certain spasticity, but the abnormal 
spinal reflexes above mentioned are no longer present. 



SYSTEMATIC DIAGNOSIS I49 



SYPHILITIC MUSCULAR ATROPHY, probably 
due either to spinal parenchymal lesions, or to root 
neuritis, or to both. 



Case 33. Joseph Graham, now 50 years of age, seemed 
no longer to be able to do good work as a teamster. His 
arms had become weak and the muscles had become tremulous 
and apparently wasted. There was also pain in the left leg 
and hip. It appears that this latter symptom had been 
thought to be rheumatism, having begun about 8 years before 
with a sudden sharp shooting pain in the left hip, about the 
region of the sciatic notch. Graham had rubbed the hip 
with liniment, but without reducing the so-called rheumatism. 
The trembling of the hands had begun some years later, 
but no wasting had been noticed except during the past year. 
The pain in the leg had suddenly become so severe that a 
month before medical observation he had quit work. The 
question immediately arose whether Graham was not 
suffering from some familial form of muscular atrophy; 
but according to his representations, there was nothing of 
the sort in the family. 

Physically, there was little to note. Neurologically, there 
was more. The pupils were somewhat irregular in outline, 
and the right was larger than the left. The left pupil failed 
to react to light, and the right pupil reacted very slowly 
and with but a slight excursion. There was no tremor of the 
tongue and no evidence of facial palsy nor was there smooth- 
ing of the nasolabial folds. It was somewhat remarkable, 
that in the absence of these signs, there was a marked speech 
defect. The atrophy of arms, forearms, and hands was 
well marked, especially the atrophy of the thenar and hypo- 
thenar eminences of the right hand. The extended hands, 
especially the right, showed a marked coarse tremor. Fibrilla- 
tion was found in the muscles of the hands, forearms, arms, 
and pectoral muscles. There was no dysmetria, and the dla- 
dochokinesia was normal. Strength was diminished (dyna- 



150 SYSTEMATIC DIAGNOSIS 

mometer right hand, 32 kg., left 31 kg.). There was little or 
no atrophy of the legs, although the left thigh was perhaps 
slightly atrophic and the gluteal muscles of the left side 
were somewhat flabby. The patellar and Achilles reflexes 
were absent on both sides. There was a slight swaying in 
Romberg position. Gait was normal. There was a marked 
tenderness on the left side of the sciatic notch, as well as 
over the entire distribution of both external and internal 
popliteal nerves. This area of skin was also hyperesthetic. 
There were no other neurological signs on systematic examina- 
tion. 

Diagnosis: The sensory disorder, the speech defect, and 
the pupillary abnormalities seem to render the diagnosis 
of progressive muscular atrophy doubtful. Nor was there 
any dissociation of sensations to suggest a syringomyelia. 
Under such circumstances, one must fall back upon the ques- 
tion of syphilis. Both blood and spinal fluid proved to be 
positive to the W. R.; the globulin was increased and the 
albumin markedly so; there were 61 cells per cmm., and the 
gold sol reaction read 4444321000. 

1. Is there a relation of Syphilitic Muscular Atrophy to 

amyotrophic lateral sclerosis? Spiller, some years since, 
claimed such a relation, and it would seem with some 
justice. 

2. How shall the present case be classified? There is 

evidence of root pains (left hip). We may naturally 
suppose that these root pains are reasonably good 
clinical evidence of a meningitic lesion, of which the 
spinal fluid clinically gave a confirmation. The fi- 
brillation in this case somewhat suggests, however, a 
central origin for the muscular atrophy. Accordingly, 
it would be difficult to definitely classify the present case 
as either one of meningovascular syphilis or one of 
central syphilis. It will be remembered that Head 
and Fearnsides classify muscular atrophy under both 
these headings. 



SYSTEMATIC DIAGNOSIS I5I 



The period of SECONDARY SYPHILIS is fre- 
quently (over a third of all cases?) MARKED BY 
approved signs of NEUROSYPHILIS precisely like 
those of full-blown paretic or diffuse (meningo- 
vascular non-paretic) neurosyphilis. These signs 
occur sometimes in association with severe clinical 
symptoms, sometimes without clinical symptoms. 



Case 34. John Bennett, 28, was brought to the Psycho- 
pathic Hospital much confused. His brother, who came 
with him, said that he had been a very heavy drinker but had 
given up drinking about four months before. He had 
recently had a cold but was otherwise In good health up to 
the night before admission. On this night, Bennett had 
become suddenly excited and went into his mother's room, 
at the common home, and began to curse her. However, he 
was put to bed safely, but on the next morning began to 
moan continuously. After some hours of moaning, he was 
brought to the hospital. Here he remained difficult to manage, 
being irritable, noisy, and resistive. Questions he either would 
not or could not answer, and there was even no evidence 
that he understood questions. However, within a few hours, 
it was clear that he was slowly coming out of the confused 
state. On the following day, it was possible even to rouse 
him and get his name. The confusion gradually cleared 
still further and, by the end of three days, he had become 
mentally absolutely well so far as could be determined. 

He then informed us that he had had a chancre about five 
or six months before, followed by a secondary skin eruption; 
that he had received four Injections of salvarsan (the last,' 
a month before admission) and three injections of mercury. 
At about the time of the last injection of salvarsan, he had 
developed headache with pain and slight stiffness in the back 
of his neck; and a fortnight later, he began to have dizzy 
spells, followed during the last week by difficulty in hearing. 
There was amnesia for everything that happened after his 



152 SYSTEMATIC DIAGNOSIS 

Spell of sudden excitement on the evening before admission, 
and this amnesia was never lifted for the four days that 
followed. 

Physically, Bennett was very well built and muscular. 
Nor were there any evidences of disease outside the nervous 
system. There was some slight stiffness of the neck and slight 
pain on movement of the head, which probably ought to be 
attributed to meningitis. The neurological examination 
showed tendon reflexes all normal, and normal sensations. 
There were, in fact, no neurological signs except that both 
pupils were dilated ; the left was larger than the right. Both 
pupils reacted to light but reacted very poorly. They re- 
acted much better to accommodation. 

The W. R. proved to be positive, as might well be ex- 
pected in a man whose infection had taken place less than 
six months before. The globulin and albumin of the cere- 
brospinal fluid were in great excess, of a degree which we 
clinically express by + + + + . The W. R. of the fluid also 
was strongly positive down to o.i of a cmm. The gold sol 
reaction was the " paretic " type, and there were 228 cells 
per cmm. 

1. How early may clinical evidence of neurosyphilis set in 

after infection? Craig found one case of " brain 
syphilis " occurring one month after infection. Frye 
claims a case of tabes dorsalis developing six weeks 
after infection. Craig states that he has had three 
cases of brain syphilis occurring within six months, 
and six within a year of infection. 

2. What effect did the salvarsan injections have in causing 

or preventing the symptoms in this case? Nonne sums 
up the neurorecidive question as follows: Since the 
jintroduction of salvarsan therapy for neurosyphilis, 
paralyses of various cranial nerves are seen more fre- 
quently. This higher frequency is in part only ap- 
parent since more attention has been paid of late to 
auditory and labyrinthine disorders. On the whole, 
however, it must be considered that salvarsan does mo- 
bilize spirochete foci which without salvarsan therapy 
would perhaps have remained latent. Probably we are 
here dealing in some instances with fresh infections of 
neurosyphilis, in other cases with a Herxheimer reaction. 



SYSTEMATIC DIAGNOSIS 1 53 

Ehrlich believed that these latent foci occur particu- 
larly in places with stagnant blood current; as, for 
instance, in the narrow bony canals. This hypothesis, 
sufficient in some instances, is less satisfactory for cases 
of peripheral neuritis, for example. 
What treatment is indicated? Intensive antisyphilitic 
treatment is strongly indicated. Whatever may be the 
truth concerning the production of neuro-recurrences 
(" neurorecidives ") it is certain that the symptoms 
usually vanish with a continuance of salvarsan therapy. 
The important point is to give efficient treatment, and 
in a case like Bennett's improvement is fairly certain 
unless some serious insult occurs before the remedial 
efforts have been given time. It is still an open ques- 
tion whether intraspinous treatment is more efficient 
in such cases than intensive intravenous injections of 
salvarsan. In Bennett's case diarsenol was injected 
intravenously twice a week in 0.6 gm. doses, reenforced 
with intramuscular injections of mercury salicylate and 
potassium iodid by mouth. Under this treatment im- 
provement began slowly and in a few months he was 
symptomatically well and after three months his tests 
were practically negative. 



154 SYSTEMATIC DIAGNOSIS 



JUVENILE PARETIC NEUROSYPHILIS (" juve- 
nile paresis ") with OPTIC ATROPHY. 



Case 35. Mary Coughlin, a blind girl of 16 years, was 
brought to the hospital in a state of great excitement, laugh- 
ing and crying alternately. The neurologist is entitled to 
think of blindness, and particularly of the optic atrophy 
which Mary showed, as probably due to syphilis. However, 
there was no history of syphilis in the father, who died in an 
accident at the age of 40, or the mother, who died at 45, of 
heart trouble. An elder sister was married and well; two 
younger sisters were living and well. The fifth sibling, a 
boy, had died in infancy. There had been no miscarriages. 
In fact, the only point in favor of syphilis was the somewhat 
far-fetched point that the younger brother of the patient 
had died In Infancy. 

The patient's history was rather suggestive of some other 
diagnosis. Her birth had been normal, she walked and 
talked at 13 months, was at school from six to twelve, reach- 
ing the seventh grade, and was considered bright. At three 
years of age, she had been run down by a car and dragged 
under the fender for a considerable distance. Her head was 
hurt but the patient did not lose consciousness In the accident. 
Fainting spells began at 11, In which spells the patient would 
lose consciousness for a minute or two. About this time, the 
patient's eyesight had begun to fail, and for some four years 
she had been entirely blind. Headaches had come on of late. 

The Coughlin case, except for the above-mentioned sus- 
picion of syphilitic optic atrophy, might be regarded as an 
unusual example of a post-traumatic disease. 

We found her to be fairly well developed and nourished; 
there was a deformity of the lower half of the sternum and of 
the third and fourth ribs on the right side. There were no 
other physical phenomena found upon systematic examina- 
tion. The left pupil still reacted to light; the right failed to 
react, but this lack of reaction could not be regarded as of 



SYSTEMATIC DIAGNOSIS 155 

Argyll- Robertson nature on account of the finding of optic 
atrophy with the ophthalmoscope. 

Mentally, it appeared that the patient's retention of 
school knowledge was poor, though her blindness for four years 
had doubtless given her little opportunity to keep such infor- 
mation fresh. Rather strangely, Mary gave utterance to 
many delusions: first, expecting to receive her sight by an 
operation on the head; second, to write a book of her doings; 
third, to buy a house for the children; fourth, would pay 
$3000 for the house, earning the money by working at a 
tailor's or as a trained nurse; fifth, to go on the stage to earn 
money by dancing; sixth, will have lots of money. 

One of Mary's characteristic statements is as follows: 
" Won't it be lovely when I can see Dr. H.'s face in heaven or 
some other lovely place? Dr. H. was a grand doctor to me, 
and when we get together again we are going to Tremont 
Temple and keep us together. I am going to do some dancing 
and play the piano. I am going to graduate at the high 
school and go to Trinity College in Washington, and I hope 
I shall be a faithful keeper of mother's tomb." 

The patient was at times euphoric and expansive. 

At this stage, what with optic atrophy, euphoria, and 
expansive delusions, we should perhaps be entitled, had Mary 
been an adult, to offer the diagnosis General Paresis. In 
fact, on the whole, any other than a syphilitic cause for the 
optic atrophy was exceedingly doubtful. Brain tumor of a 
nature to produce optic atrophy might very improbably last 
so long as five years. There was no evidence of any in- 
toxication at the time when the blindness occurred. 

The W. R. was positive in the blood and spinal fluid; 
there was a positive globulin test, and an excess albumin 
as well as 15 cells per cmm. 

1. What is the significance of Mary's trauma at three years? 

So far as we are aware, none. 

2. What light could be thrown by a W. R. study of the 

family? In some instances, much light is thrown; in 
the present case all three living sisters of the patient 
have been examined and their serum W. R.'s have been 
found negative. 



156 SYSTEMATIC DIAGNOSIS 

3. What is the prognosis of juvenile general paresis? Death 

within a few years, as in general paresis in adults. 
The patients live rarely more than four or five years after 
the onset of symptoms. Mary Coughlin died a year 
and a half after the above examination, namely, in her 
eighteenth year, some seven years after the onset of 
symptoms. 

4. What can be said of treatment? A few favorable results 

have been reported after intraspinous therapy (Swift- 
Ellis). Too little work has been done with systematic 
treatment of juvenile neurosyphilis, both paretic and 
non-paretic, to permit important conclusions at this 
time. 

5. How can we explain the infection of this sibling whereas 

the others, both younger and older, escaped? It would 
seem that we would have to discard the hypothesis of 
a congenital infection and consider that it was acquired 
accidentally during the lifetime of the patient. Con- 
sidering the prevalence of syphilis it is rather to be 
wondered that more such cases of " innocent " infection 
do not occur in children. We may recall how many 
instances of juvenile gonorrhea occur. In a case as 
this where the symptoms calling attention to syphilis 
necessarily occur so long after the original infection it 
is practically impossible to trace the origin of the 
infection. 



SYSTEMATIC DIAGNOSIS 1 57 



The diagnosis of JUVENILE PARESIS is often 

easy. 



Case 36. Theresa Mullen, an under-sized girl of 12 years, < 
presented a remarkable appearance due to congenital am- 
putations of the fingers and toes. She lay in bed, drivelling 
and making unintelligible cries. It appeared that the patient 
weighed about 12 pounds at birth and was very fat; that 
she had been fed on condensed milk, had survived cholera 
infantum, whooping cough, and, as the parents said, ** two 
kinds of measles." 

Theresa had gone to school at 5 years, reaching the third 
grade at the age of 9; but at this time, she began to lose 
ground and was put in a class for backward children. More- 
over, at about this time, the teachers noticed spells of cause- 
less laughter and meaningless twisting back and forth. 
Theresa would also scream at night, looking about the room ; 
once, rising and crying, " Take him away, that black thing," 
though no appropriate object was present. There had been 
little or no complaint of headache. Theresa had been de- 
teriorating for some time, and for a year past had been 
having increased difficulty in walking. For two months the 
child had not spoken Intelligible words; for the last week, 
she had been incontinent. 

The diagnosis was almost obvious from the manual and 
pedal deformities taken in connection with the saddle-back 
deformity of the nose. It was interesting in connection with 
the contentions of W. W. Graves, that the scapulae were 
scaphoid In type. 

Accordingly, the history given by the parents seemed 
consistent enough. The parents were both 36 years of age, 
having married at 23. The first pregnancy was a miscarriage 
at two. months, of unknown cause. Theresa came next; 
thirdly, came a miscarriage at three months; fourthly, a 
girl, who Is not strong or well physically, has suffered much 
from headaches and sore throat, but is fairly bright. The 



158 SYSTEMATIC DIAGNOSIS 

fifth pregnancy resulted in a boy, who is bright but of under- 
size. Three more pregnancies resulted in miscarriage. 

Taking into account the above-mentioned physical charac- 
teristics, the personal history, and the family history of 
Theresa, the diagnosis could hardly be in doubt even in the 
absence of a lack of pupillary reaction to light on the right 
side, infantilism of genitalia, positive W. R.'s of serum and 
spinal fluid, positive globulin, and excess albumin, 34 cells 
per cmm. and the paretic type of gold sol reaction which 
were found. 

The prognosis of this case appears to be rapid deterioration, 
terminating in death within a few months. Now and again, 
however, some such cases spontaneously improve. Such a 
case as that of Theresa Mullen is always disheartening in 
itself but suggests the social value of Wassermann tests in the 
other members of the family. The other children of the Mul- 
len family proved to be suffering also from syphilis, since 
their blood sera all showed a positive W. R. 

1. What is the characteristic age of onset in Juvenile 

Paresis? An impression has prevailed in some quarters 
that the typical onset of juvenile paresis is in the ado- 
lescent years, and Clouston's first case (1877) developed 
in a boy of 16. Thierry's 58 cases, developing from the 
8th to the 20th year, averaged 14 years of age at onset. 
Mott's 22 cases from the 8th to the 23d year, averaged 
17 years at onset. According to Clouston, juvenile 
paresis develops most often at puberty (15 to 17 years). 
It is sometimes claimed that cases developing symptoms 
early live longer, and that juvenile cases developing 
symptoms after the 20th year run a short course. For 
a case developing in the 5th year, see John Friedreich, 
Case No. 77. 

2. What may be concluded from the physical signs (con- 

genital amputations) present in this case before the 
development of mental symptoms? Some cases of 
juvenile paresis appear to show no physical signs what- 
ever in childhood. While these amputations might be 
the accidental result of a difficult delivery, it is more 
probable that they are due to a syphilitic process. 



<r 




Juvenile paresis — congenital amputation of digits. This 
case reached fourth grade in school before deterioration. 



SYSTEMATIC DIAGNOSIS 1 59 



CONGENITAL SYPHILIS is apparently capable 
of producing simple FEEBLEMINDEDNESS (that 
is, a form of disease non-paretic, non-tabetic, with- 
out Special tendency to progression, and without 
tendency to vascular insults). 



Case 37. Isaac Goldstein was a small boy of six years and 
seven months, with a father known to be suffering from gen- 
eral paresis. The child was very irritable and nervous and 
very difficult to manage, but would hardly have been the 
subject of medical attention except in a family study sug- 
gested by the paresis of the father. 

The child had been born at term and had apparently 
undergone a normal development. Physically, he showed no 
definite signs of congenital syphilis. In fact, the physical 
examination was to all Intents and purposes negative. The 
W. R. of the serum, however, proved to be positive. Mental 
tests showed that his mental age was that of a child of a 
little over five years. Taking all things into account. It Is 
probable that he should be regarded, therefore, as somewhat 
retarded mentally. 

I. Is syphilis answerable for the mental retardation In this 
case? Provided that the family is free from feeble- 
mindedness and mental disease, It would seem that 
the retardation of a congenital syphilitic should per- 
haps be regarded as syphilitic in origin. Of course, the 
institutions for the feebleminded have not shown ex- 
ceedingly high percentages of syphilitic children in 
various W. R. surveys; still, the percentage of positive 
reactions in institutions for the feebleminded is clearly 
higher than the incidence of congenital syphilis shown 
in the population at large. Hence, we may conclude 
that syphilis is one of the etiological factors in the 
production of feeblemindedness. Dr. W. E. Fernald, 
of the Waverley School for the Feebleminded, has re- 
cently pointed out that the syphilitic cases belong 
rather in the lower grades (Idiots and Imbeciles) of 
feeblemindedness than in the higher (morons). 



l60 SYSTEMATIC DIAGNOSIS 

2. Can we guess what the pathological anatomy and histol- 

ogy of the brain may be in such cases? The Waverley 
studies now in process seem to indicate that some 
cases have little or no gross alterations, but show a few 
slight traces of lymphocytic accumulations discovered 
upon extended search, and a certain tendency to the 
appearance of rod cells in various foci. But the whole 
matter is still suh judice. It is a question whether 
these traces of chronic inflammation are the residuals of 
a more active process or the beginnings of a process that 
is about to be more active. 

3. How characteristic is a positive W. R. in the serum of a 

child without physical stigmata of congenital syphilis? 
If we limit the term stigmata to the major and more 
important signs, we must reply that it is not unusual 
to find positive W. R.'s in sera of physically nor- 
mal looking children. Except in family studies, such 
cases will often escape notice, either because there 
are no stigmata whatever, or because such stigmata 
as exist are of a minor nature and regarded as unim- 
portant anomalies. Some of these cases occur in the 
clinics later in life as so-called syphilis hereditaria tarda. 
If one wishes to discover these cases with late de- 
velopment of symptoms before their full bloom, the 
most obvious method is to examine carefully the chil- 
dren of known syphilitics. 




Scaphoid Scapulae. 



SYSTEMATIC DIAGNOSIS l6l 



nile tabes ") ; TREATMENT. 



Case 38. The point in presenting Archibald Sherry, a 
Juvenile Tabetic of 12 years on admission, is perhaps 
to exhibit pride in therapeutic results. 

There was little or no doubt of the diagnosis ; in an adult, 
the phenomenon would be called tabes dorsalis with a ques- 
tion of general paresis. The right pupil was larger than the 
left and reacted neither to light nor to distance. There was 
a slight tremor of the tongue and of the outstretched hands. 
The knee-jerks and ankle-jerks could not be obtained, nor 
could the periosteal reflexes in the legs. There was a slight 
unsteadiness in the gait and in various finer movements, and 
a slight ataxia of the legs. There was not a classical Romberg 
sign but there was slight swaying in Romberg position. The 
teeth were Hutchinsonian. For the rest, the physical ex- 
amination was practically negative. I 

The family history was of interest. On the paternal side 
there was nervousness as well as alcoholism and degeneracy. 
The maternal grandmother had cancer. Archibald's father 
was immoral and alcoholic. There was a girl four years 
older than Archibald, who, though nervous and unstable, has 
shown no signs or symptoms of syphilis and does not yield a 
W. R. in blood or spinal fluid. 

Archibald himself was bom at term, a large child, who, 
however, lost weight rapidly, developing a marked skin 
eruption on head and back three weeks after birth. This 
skin disease lasted for a month and a half and then spon- 
taneously disappeared. Archibald remained weak and sickly, 
not walking until three years of age. However, he did well 
in school up to the end of his nth year, when he failed to 
keep up with the children. He had been an amiable child 
and had gotten on well with his playmates. Some time in 
his loth year physical disability had begun; there was numb- 
ness in the legs with weakness; at times, actual inability to 



I 



1 62 SYSTEMATIC DIAGNOSIS 

walk. The right pupil was noticed by the mother to have 
increased in size; the eyelashes had turned white. There 
was pain over the left eye and a feeling of weight on top of 
the head. Speech became difficult or even confused. 

Consistently enough, the W. R. both in blood and spinal 
fluid was positive. Globulin and albumin were present in 
large amounts; there were 150 cells per cmm. 

Granting that this be in some sense a case of juvenile 
tabes we may raise a doubt whether the case is one of congeni- 
tal syphilis. The W. R.'s of the blood of both father and 
mother are negative. Syphilis is denied by them. The 
nervous and unstable older sister failed to show definite 
symptoms of syphilis or a positive W. R. There had been no 
miscarriages or stillbirths. The question arises whether the 
Hutchinsonian teeth do not indicate congenital syphilis. It 
appears, however, that it is possible to develop Hutchinsonian 
teeth if syphilis is acquired before the teeth are formed. We 
have no data as to how or why this particular baby should 
have acquired syphilis, if he did so acquire it, at the age of 
three weeks. On the whole, sceptics may doubt our sug- 
gestion that the case is one of acquired juvenile tabes. Pos- 
sibly the question is academic so far as treatment is concerned. 

Prognosis: The rarity of juvenile tabes is such that little 
can be said as to prognosis. Three and a half years have 
passed since a few injections of salvarsan were made. The 
pains above mentioned rapidly disappeared, the gait became 
steadier, the attacks of confusion ceased, and the speech im- 
proved. Unfortunately, on account of a lack of cooperation 
on the part of Archibald's mother, we have been unable to 
continue treatment. However, we have from time to time 
followed the patient in his home and he seems to have shown 
no falling back after the initial improvement. It would be of 
great value could we know the situation in the spinal fluid at 
the present time. 

I. Is there any explanation why paresis should occur in 
some juveniles and tabes in others? There is no 
available explanation for this difference nor any for 
the characteristic early optic atrophy of juvenile 
tabetics. 



Be frustrate, all ye stratagems of Hell, 
And, devilish machinations, come to nought! 



Paradise Regained, lines 180-181 



III. PUZZLES AND ERRORS IN THE 
DIAGNOSIS OF NEUROSYPHILIS 

This part of the case collection, dealing with puzzles and 
errors, Is ushered In by six cases (39-44) drawn from a group 
of errors In diagnosis made some years since at the Danvers 
Hospital. These six are autopsied cases. Attention Is called 
to the fact that modern methods of diagnosis might have 
prevented the errors. 



DIFFUSE NEUROSYPHILIS (" cerebrospinal 
syphilis") versus PARETIC NEUROSYPHILIS 
(** general paresis ") . Autopsy. 



Case 39. Caroline Davis, dead at 49 years, was a case 
of error In the diagnosis of general paresis. Like Cases 
40 to 44, Case 39 was diagnosticated by the full Danvers 
staff as a case of general paresis ; however. It must be added, 
before the days of the W. R. and the modern methods of 
systematic diagnosis. As will transpire In the sequel, there 
is a large question whether Case 39 is not after all really a 
case of neurosyphilis, possibly not of the paretic group. 
The details are as follows: 

Caroline Davis was a normal school girl till 15, apt in 
studies, mill worker till marriage at 18; one child, dead 
(cause unknown). Habits good. Moderate deafness set in 
in the forties and in 1901 patient became completely deaf 
in three months' time. In 1905 she became unable to take 
care of her house and had a shock in which the right leg was 
affected. 

On commitment patient showed good development and 
nutrition with slight enlargement of capillaries of cheeks, 
redness and roughening of skin of right ankle. Teeth 

165 



1 66 PUZZLES AND ERRORS 

absent. Slight radial and brachial arteriosclerosis. Urine 
negative. Sluggish pupil reactions to light both directly and 
consensually. Deafness absolute, bone conduction defective. 
Arm reflexes brisk, knee-jerks equal, brisk. Bilateral Ba- 
binski reaction more marked on the right side, tremor of 
tongue, Romberg's sign, gait defective. Speech stumbling, 
writing clear, without tremor. 

Communicated by writing only. Consciousness normal, 
disorientation for day of month, for place (misnames hospital) 
and for persons (recognizing nurses, not patients). 

Patient wrote many letters complaining of pain, headaches 
and especially of pain in the abdomen and side. The patient 
was thought to show a slight defect of memory, but her 
deafness rendered diagnosis difficult. The patient died 
suddenly on May 23, 1908, shortly after supper, falling back- 
wards, and dying in five minutes with marked respiratory 
distress. 

Post Mortem Findings. The cause of death was not clear. 
The heart's blood and cerebrospinal fluid were sterile. There 
was a small hemorrhage in the anterior part of the right 
ventricle derived from a small artery of the caudate nucleus. 
There was about 400 cc. of blood between the dura mater and 
the pla mater. There was a slight sclerosis of the basal and 
Sylvian arteries. The brain substance was uniformly softer 
than normal. 

It is possible that the hemorrhage had taken place some 
time before the patient's fall and that the brain substance 
had swollen in consequence. Just before the fall she had a 
weeping spell. 

The anatomical diagnoses were as follows : 

Obesity, unequal pupils, fresh wound near left ear, edema 
of legs, slight focal adhesive pleuritis, hypostatic congestion 
of lungs, chronic endocarditis, chronic myocarditis, congestion 
of kidneys, congestion of pancreas, subacute splenitis, chronic 
adhesive pelvic peritonitis, hematoma and cystic condition of 
Fallopian tubes, calvarium dense and thick, subdural hem- 
orrhage, slight chronic leptomeningitis, general cerebral at- 
rophy, marked in tips of frontal lobes, old cyst of softening 
between left corpora albicantia and optic chiasm, small 



PUZZLES AND ERRORS 1 67 

punctures of left ear drum, drums opaque, chronic spinal 
leptomeningitis; brain weight, 11 90 grams. 

There were marked firm interadhesions between dura and 
pia throughout. A lumbar puncture soon after admission 
in 1907 had shown: 

Per cent 

Endothelial cells 10 

Lymphocytes 30 

Plasma cells o 

Phagocytes o 

Polymorphonuclear cells 51 

Unclassified 9 

Fibroblasts o 

Cells in 100 fields 125 

It will be noted that the lumbar puncture yielded no 
plasma cells and yet showed 30% of lymphocytes. Alz- 
heimer, in 1904, attempted to distinguish the histology of the 
cerebral syphilitic from that of the general paretic, main- 
taining that lymphocytosis was the characteristic feature of the 
ordinary neurosyphilitic, whereas plasma cells were associated 
with the lymphocytes in the paretic. This case showed lym- 
phocytic deposits. To be sure, they were decidedly sub- 
ordinate in the cerebral cortex, cerebellum, and basal ganglia, 
to the marked evidences of nerve cell destruction, although 
there were perivascular infiltrations about a few of the larger 
vessels in the white matter of the cerebral cortex. 

The spinal cord, however, showed a most severe Infiltra- 
tion, especially in the gray matter, where the Infiltration 
accompanied severe nerve cell changes and arterial changes. 
The pia mater of the spinal cord was also packed with mono- 
nuclear elements, among which, however, no plasma cells 
could be found. 

But although the inflammatory changes In the shape of 
lymphocytosis were relatively more prominent In the spinal 
cord than in the cortex, yet the cortex yielded evidence of an 
exceedingly marked destructive process. Perhaps no layer 
of any of the areas of the cortex examined failed to show some 
atrophic alteration. The upper layers of the cortex were 
everywhere more severely diseased than the lower layers. 
Here we are dealing with an instance of an active meningomye- 



1 68 PUZZLES AND ERRORS 

litis and subcortical encephalitis. It is, of course, probable 
that the W. R., had it been performed, would have been 
positive in this case. On the basis of the histology, we are 
inclined to regard the clinical picture in this case as belonging 
among cases of Non-paretic Diffuse Neurosyphilis. 

This case, as also the next several, is especially instructive 
in teaching the difficulty in differentiating paretic and non- 
paretic neurosyphilis. Not only is this difficulty met in 
clinical diagnosis, but in pathological diagnosis as well. 

The histological diagnosis depends in large part on the 
work of the Nissl- Alzheimer school, which has received great 
recognition. At the present time, however, there Is begin- 
ning to be considerable doubt as to the entire validity of this 
teaching. At any rate there are many borderline cases in 
which the differentiation is well nigh impossible. In this 
case note chronic meningoencephalitis, with cortical degener- 
ation, in the absence of plasmocytosls. 

From the clinical standpoint the Intensity of the W. R., 
the character of the gold sol reaction, and the result of 
therapy have added new points in differentiation. Much 
more work controlled by autopsies Is still needed, however, 
to put us on sure ground in borderline cases. 



PUZZLES AND ERRORS 



169 



VASCULAR NEUROSYPHILIS (?) versus PARETIC 
NEUROSYPHILIS (" general paresis "). Autopsy, 



Case 40. Case 40 like Case 41 was an error in the diag- 
nosis of general paresis which might be regarded as academic 
rather than practical. Both were cases of arteriosclerotic 
brain disease with severe cerebellar involvement. Case 40 
had a spinal cord that was not quite normal. There was a 
tabetiform lesion In the cervical spinal cord (not elsewhere), 
together with a unilateral degeneration suggesting in some 
respects a radicular origin. The most striking feature, how- 
ever, of Case 40 as in Case 41, was a lesion of the cerebellum. 
In Case 40 the dentate nuclei were in large part destroyed 
by cysts of softening, although the cerebellar cortex was 
fairly well preserved on both sides. The details of Case 
40 are as follows: 

H. F., male, gear maker, born 1850. 

Heredity. Maternal grandmother insane. Mother insane 
at 52, became demented and lost use of limbs, died at 71. 
Aunt insane. 

Personal History. Common school education. Capable 
workman till within a few months. Early in life alcoholic. 
Drunk almost every week until 1899 or 1900. Irritable, 
nervous, selfish, loose in relations with women. Venereal 
disease denied by wife. Married in 1883. Three frail 
children. No miscarriages. Neuralgia in 190 1 or 1902. 

January, 1904, patient left carriage shop on account of 
mistakes in work, became more pleasant, childish, fearful, 
talkative, did funny things, later became vagrant, stole from 
fruit stores, smoked cigarettes picked up in the street, and 
became restless and irritable. 

Committed to Danvers, June 24, 1904, with slightly 
enlarged heart, somewhat heightened blood pressure, and a 
slight sediment of epithelial cells in urine. 

Romberg's sign was present, but there was little or no 
demonstrable incoordination otherwise. Very slight tremor 



170 PUZZLES AND ERRORS 

of fingers. Left knee-jerk absent, right obtained on re- 
enforcement. Achilles jerk absent. Triceps, wrist and 
normal plantar reflexes present. Pupils react to accommoda- 
tion, but very slightly, if at all, to light. Sensations normal 
except in legs. The legs show preservation of tactile and 
temperature senses, but abolition of pain sense except over 
dorsum of foot. 

Speech showed slurring of syllables and " brigrade " for 
" brigade." Disorientation for time, place and in part for 
persons. Admitted that his work had been deficient but 
regarded himself as well. Emotionally variable, crying at 
times and suddenly becoming jocular. Eloped July 3 and 
somehow reached his wife's house in a neighboring city. 

Euphoria persisted. The pupils continued Argyll- Robert- 
son, and the knee-jerks remained absent. Became oriented 
for place and partially as to time (month"and day of week 
correct) . 

During 1905 failure became rapid, with ataxia of legs, 
persistent euphoria, and loss of weight. 

Convulsions, regarded as general paretic, developed in 
1906. Death sudden, December 7, 1906. 

Post Mortem Findings. The cause of death was strep- 
tococcus septicemia, probably derived from a gangrenous 
bronchopneumonia or related with a small thrombus of the 
right auricular appendix. There was also an acute purulent 
otitis media, mastoiditis and sphenoidal sinusitis, as well as 
extensive decubitus. From this decubitus or from the 
intestinal tract may have been derived the numerous colonies 
of bacillus coli communis which developed on plates from the 
cerebrospinal fluid. 

Arteriosclerosis was little in evidence, being confined to 
the coronary, right vertebral and carotid arteries (slight in 
all). Cysts of softening existed in the posterior part of each 
dentate nucleus and may probably be interpreted as indicating 
vascular disease. 

Chronic disease outside the nervous system was prominent 
and in part suggestive of senile findings; milky patches of 
pericardium, adhesions about liver and gall-bladder, adhesions 
about spleen, adhesions and fibrous thickening of parietal 



PUZZLES AND ERRORS I7I 

peritoneum, adhesions in both pleural cavities, chronic diffuse 
nephritis, hypertrophy of bladder wall, dense calvarium, dural 
adhesions. 

The nervous system showed several unexpected features. 
The absence of chronic leptomeningitis was striking: the pia 
mater was everywhere delicate and transparent except that 
the walls of the cerebellar and chiasmal cisternae were thick- 
ened and that there were slight opacities along the sulcal 
veins of the convexity. Brain weight 1090 grams. There 
was a generalized sclerosis and pigmentation of the cerebral 
cortex. The sclerosis varied in degree and was most marked 
in the prefrontal regions, the anterior halves of the superior 
frontal gyri, the middle third of the right precentral gyrus, 
the region of the splenium on the left side, and the sagittal 
rami. If the bacillus coli communis found in the cerebro- 
spinal fluid had any effect upon the consistence of the brain, 
obviously hard to prove in a brain of leathery consistence at 
the outset, it was shown only in the right Rolandic area in the 
vicinity of the sclerotic part of the precentral gyrus. Granu- 
lar ependjrmitis of all ventricles. Weight of cerebellum, pons 
and bulb, 135 grams. 

Perhaps the most remarkable feature of all in the case was 
the occurrence of cysts of softening in the posterior part of 
each dentate nucleus. For discussion, see Case 41. 



172 PUZZLES AND ERRORS 



VASCULAR NEUROSYPHILIS (?) versus 
PARETIC NEUROSYPHILIS (" general paresis »). 
Autopsy. 



Case 41, like Case 40, was one of arteriosclerotic brain 
disease with severe cerebellar involvement. Here is another 
case in which the Danvers staff made a diagnosis of general 
paresis without dissenting voice. There were some tabetic 
symptoms, and the spinal cord at autopsy did show a moderate 
lymphocytic infiltration of the meninges, entirely consistent 
with the picture in the spinal fluid. In this case, the dentate 
nuclei of the cerebellum were not destroyed as in Case 40, 
but were affected by cell atrophies of variable degree in differ- 
ent parts of the nuclei. There was also a severe gliosis of 
the cerebellar cortex. The left hemisphere of the cerebellum 
was more severely diseased than the right. The cortex 
showed far more marked and generalized cell atrophies 
throughout the layers than did Case 40. The details of this 
case, which was that of a colored coachman, Samuel North, 
are as follows: 

He was born in 187 1. Learned to read and write at school. 
Stableman and coachman. Alcoholic till 1902. Took much 
quinine, possibly impairing hearing thereby. Memory im- 
paired and growing worse since 1902. Gait unsteady for a 
longer but unknown period. August 13, 1907, wandered 
about, instead of attending boot-black stand, muttered, 
talked incoherently. In the next few days talked about 
religion and apparently had hallucinations of hearing. Com- 
mitted August 16, 1907. 

On commitment stoop-shouldered, flat-chested. Gait stag- 
gering. Unsteadiness in Romberg's position. Incoordina- 
tion of arms and fingers. Coarse tremor of tongue. Tremor 
of lower jaw. Exaggeration of left knee-jerk and diminution 
of right. Exaggerated Achilles jerks. Spurious left ankle 
clonus. Questionable Babinski reaction of left side. Ab- 
dominal and epigastric reflexes present but cremasteric 



PUZZLES AND ERRORS 1 73 

absent. Left pupil smaller than right and fails to react to 
light. Reaction of right pupil sluggish. Moderate defect of 
hearing of both sides. 

During the first week the patient developed hallucinations 
of sight and hearing, but of no other senses. Disorientation 
for time, place, and persons. Answers to arithmetical 
problems given with assurance but as a rule incorrectly (as 
17 and 32 are 90; 18 divided by 3 is 88). Handwriting 
scarcely legible. Memory poor, especially for recent events 
(recalled a lumbar puncture as an exercise in baptism). 
Impressibility and attention poor. Euphoria. 

Death after gradual failure July 29, 1908. 

Lumbar puncture showed : Per cent. 

Endothelial cells 9 

Lymphocytes 81 

Plasma cells 6 

Phagocytes o 

Polymorphonuclear cells 4 

Unclassified o 

Fibroblasts o 

Cells in 100 fields 700 

Post Mortem Findings. The cerebrospinal fluid showed 
a pure culture of Bacillus coli communis, and the heart's blood 
showed many colonies of an unidentified bacillus. Culture 
from mesenteric lymph nodes sterile. 

The cause of death is somewhat in doubt. There was an 
early pneumonic process with fibrinous pleurisy, and there 
was an early acute hemorrhagic ileitis with a very slight 
overlying peritonitis and slight corresponding enlargement of 
mesenteric lymph nodes. There was an infection of the 
meninges with Bacillus coli communis. 

Evidences of chronic disease outside the nervous system 
were: coronary and pulmonary arteriosclerosis, chronic 
fibrous endocarditis, mitral sclerosis, aortic sclerosis with 
calcification, chronic splenitis, chronic interstitial nephritis, 
hepatic atrophy (wt., 900 grams), thickening of cartilaginous 
portion of right auricle (old trauma) , scars of apices of lungs. 

The calvarium was dense and the dura mater everywhere 
adherent. The arachnoidal villi were but slightly developed, 



174 PUZZLES AND ERRORS 

but there was one small focus of cortical herniation through 
the dura mater of the left middle cranial fossa. The pia 
mater was delicate except for slight opacities along sulci. 
There was some pial thickening over the region of the inter- 
parietal sulci on both sides. There was pial pigmentation 
anteriorly and superiorly. 

There is no gross evidence of intracranial arteriosclerosis, 
except (i) that afforded by the lesions of the dentate nuclei 
of the cerebellum mentioned below and (2) the swerving to 
the right of the basilar artery, possibly due not to arterio- 
sclerotic lengthening of the artery but to an unusual shape of 
the pons (see below). 

The brain weighed 1245 grams (cerebellum and pons 165 
grams) . The anatomical diagnoses of central nervous system 
were: 

Slight general encephalomalacia (post mortem imbibition 
of fluid, 31 hours). Slight gliosis of right prefrontal and 
frontal gyri. Slight gliosis of right optic thalamus. General- 
ized granular ependymitis, especially near fornix and about 
foramina of Monro. Anomaly of pons (not gliotic, but 
possessing far more white matter on the left side than the 
right). Severe arteriosclerosis confined to the dentate nuclei 
of the cerebellum. 

As we now look over the data in Cases 40 and 41 we are 
inclined to ask the question, whether modern systematic 
diagnosis would not have shown these cases to be Neurosyphi- 
LlTic? One is inclined to answer this question in the affirma- 
tive, on the basis that Case 40 showed somewhat questionable 
Argyll- Robertson pupils, and Case 41 showed unilateral 
Argyll-Robertson effect. Both cases showed Romberg sign, 
but the dentate nucleus and other cerebellar disease in each 
case may in some way have contributed to or imitated this 
phenomenon. Whether Case 40 was a tabetic must remain 
a question, but Case 41 must be regarded as a case with spinal 
and meningeal changes highly characteristic of syphilis. 



PUZZLES AND ERRORS 1 75 



VASCULAR NEUROSYPHILIS plus TABETIC 
NEUROSYPHILIS (*' tabes dorsalis ") simulating 
paretic neurosyphilis (** general paresis "). Au- 
topsy. 



Case 42. The case of Elizabeth Brown was at one time 
carefully studied by Dr. A. M. Barrett In his work on mental 
diseases associated with cerebral arteriosclerosis and, like 
Case 43, was one in which tabes dorsalis was a factor. Eliza- 
beth Brown's maternal grandfather and mother were insane; 
there had also been insanity in a sister. Mrs. Brown was 
struck on the head at 44, and was unconscious for an 
hour, but there were no sequelae to this accident. At 48, 
there was a shock, or apoplectiform attack, followed by 
unconsciousness for two hours and by left hemiplegia, right 
ptosis, and thick speech. Mrs. Brown began to walk again 
after two weeks, but was found to be forgetful and fabulatory. 
She seemed at times to be hearing music, and somewhat 
repeatedly became helpless and unable to walk. She could 
not remember from day to day, showed Incontinence of urine 
and feces, and was brought to the Danvers Hospital. The 
physical and mental deterioration was progressive. There 
were some signs of organic brain disease. The musculature 
was especially flabby on the left side. The left angle of the 
mouth drooped, and the left nasolabial fold was smoothed 
out. The arm movements were ataxic, the tongue protruded 
to the left, the right pupil reacted but slightly to light (eye 
blind from cataract), the knee-jerks, Achilles, wrist, and 
elbow reflexes, were absent. The patient was unable to 
stand, and there was a marked tremor of the hand, tongue, 
and lips. There was a zone of anesthesia for pain and 
tactile stimulation extending round the body, from the 3d 
to the 6th rib, and there were symmetrical areas of anesthesia 
on the inner surface of the forearms and the legs. 

The autopsy showed a general arteriosclerosis with chronic 
and acute meningitis. The brain weighed mo grams; 



176 PUZZLES AND ERRORS 

the pia mater was moderately thickened; the basal vessels 
were highly arteriosclerotic. The brain itself, however, 
normal externally, upon dissection, showed a number of 
small cysts irregularly scattered in the white substance. 
The basal ganglia were porous, and there were several small 
cysts in the pons. Microscopically, there was evidence of 
severe vascular disease, involving not only the arteries but 
also the veins. It was the superficial rather than the deep 
arteries that were more often attacked. There was a marked 
perivascular gliosis. Extensive search yielded no evidence 
of lymphocyte infiltrations, either in the brain or in the spinal 
cord. 

The spinal cord showed degenerations in both the lateral 
and posterior columns, of which the explanation may possibly 
be like that in our paradigm, Case i. 

Is the case of Elizabeth Brown one of neurosyphilis? We 
cannot definitely say on account of the non-availability of 
the modern systematic tests, but it may well be that the case, 
although certainly not one of paretic neurosyphilis, was one 
of Tabes with Vascular Complications. 



PUZZLES AND ERRORS 1 77 



TABETIC NEUROSYPHILIS (" tabes dorsalis ") 
with sjmiptoms of cerebral origin producing a pic- 
ture resembling taboparetic neurosyphilis (" tabo- 
paresis "). Autopsy. 



Case 43. Robert Allen was the fifth case of error in the 
diagnosis of general paresis analyzed some years since from 
the staff meeting records of the Danvers Hospital. The 
Allen case resembles the case of Elizabeth Brown in that 
there was a combination of tabetic phenomena with cerebral 
lesions of a non-paretic character at autopsy. But al- 
though there seemed to be an utter absence of inflammatory 
cells (lymphocytosis) in the case of Elizabeth Brown (42), 
there were some slight perivascular cell accumulations in 
the Allen case, with a few mononuclear cells suggestive of 
lymphocytes. The cerebrum, however, failed to show plas- 
mocytosls. It was seriously diseased, showing a marked 
neuroglia proliferation about the atrophic nerve cells. 

Robert Allen was a printer coming from a long-lived race. 
The following are the main facts: 

Married in 1875 (two children, healthy); again married in 
1893 (one child, healthy). Compositor from 1890. In 1898 
and 1899 girdle and lancinating pains. Thereafter for 
several years gait was unsteady. During 1904 and 1905 
freedom from pains and improvement in gait but gradually 
increasing irritability and nervousness. Stopped work on 
last of IV? j.rch, 1905, owing to sudden increase of irritability, 
emotionality, boastfulness, expansive schemes, and ataxia. 

Habits: no tobacco, very little alcohol at long intervals. 
No drug habits, no sexual irregularity known. 

Committed to Danvers April 3, 1905, with slight muscular 
development, poor nutrition, acne, irregular, poorly preserved 
teeth, gingivitis, flat-foot, slight radial arteriosclerosis, slight 
arcus senilis, a few hyaline casts, leucocytes, epithelial cells, 
and trace of albumin in the urine, scar in sulcus, and enlarged 
inguinal lymph nodes. 



178 PUZZLES AND ERRORS 

Ataxic gait, Romberg's sign, fibrillary twitching of chest, 
abdominal and facial muscles when standing; right pupil 
slightly larger than left, pupillary margins irregular, light 
reactions (electric bulb test) both consensual and direct 
absent, slight pupillary reaction in accommodation; biceps, 
triceps and wrist reflexes lively and equal; abdominal, cre- 
masteric and plantar reflexes normal, knee-jerks, Achilles 
and front taps negative even on reenforcement. 

The patient himself stated that his ataxia began in 1904, 
that he had been under treatment for swelling of legs and 
feet and pain in limbs since 1903, and that there had been 
some trouble with limbs since 1895. He had been told that 
his disease was lead-poisoning. About three weeks before 
commitment patient said he had had an attack of uncon- 
sciousness. 

The patient's speech showed considerable defect. Words 
were pronounced slowly with slurring and tripping especially 
of the labials. Orientation perfect. School knowledge well 
retained. The easier arithmetical problems were accurately 
performed. Memory imperfect for minor recent events. 
Estimations of space and time often very imperfect. Vari- 
ability of mood, sometimes euphoric, sometimes tearful and 
irritable. Occasional expansive estimates of personal powers 
("Can lift three five-hundred pound weights with one finger"). 
Indistinct expansive financial ideas. 

The patient continued oriented, euphoric, expansive, 
untidy, till October, 1905, but on October 12 developed an 
infection at the site of a callus on the sole of the foot and died 
with pyemic symptoms, October 17. 

Post Mortem Findings. The cause of death was strepto- 
coccus septicemia with acute ulcerative colitis, acute splenitis, 
bilateral purulent pleuritis, multiple infarctions of lungs. 

There were no signs of chronic disease outside the nervous 
system except a moderate thickening of the mitral valves, 
and slight dural adhesions. 

The brain weighed 1450 grams. The vessels at the base 
showed a slight degree of sclerosis. There was a slight 
opacity of the frontal, parietal, and temporal pia overlying 
slightly atrophied convolutions, whose surfaces showed in a 



PUZZLES AND ERRORS 1 79 

few places slight cuppings. The ependyma over the thalami 
and the floor of the fourth ventricle was finely roughened. 
The spinal cord showed a typical Tabes Dorsalis. 

Although we probably cannot regard either Case 42 or 
Case 43 as a case of paretic neurosyphilis, and although it 
must remain doubtful whether they are cases of any form 
whatever of neurosyphilis (in the absence of the modern tests), 
yet it seems clear that both these cases may very well have 
been cases of neurosyphilis on account of the existence of a 
definite tabetic process in each. The symptoms of these 
cases, like those of Cases 38 to 41, suggest how difficult it 
must be to make a clinical diagnosis of general paresis safely 
without employing available laboratory tests. Yet how fre- 
quently in the past have neurologists brought data con- 
cerning various phenomena in long series of so-called 
paretics in which the error of diagnosis was certainly between 
5 and 15% and frequently still greater. The entire question 
of the symptomatology of paretic and non-paretic neurosyphi- 
lis, therefore, needs re-opening and revision. 



l80 PUZZLES AND ERRORS 



CEREBRAL GLIOSIS (probably non-syphilitic) 
producing the clinical picture of paretic neuros3rph- 
ilis (" general paresis ")• Autopsy. 



Case 44. John Hughes was a hostler, and later assistant 
with a wholesale drug company, with which he remained 
for 32 years. He had been moderately but constantly al- 
coholic all his adult life up to 50 years of age, and at 45 had 
had an attack of so-called nervous prostration, in which his 
head had troubled him and he had been seclusive. At 49, 
he had a serious attack of otitis media, associated with 
delirium, swelling of the feet, and what was called rheuma- 
tism. After this attack of otitis media, Hughes appears to 
have been not altogether right. 

At 53, after a quarrel with his employer, Hughes quit 
work, began to trade a little in hens and pigs, became for- 
getful, especially of recent events, and did " a variety of 
peculiar things." He was a married man but he had no 
children. There had been miscarriages but of unknown 
origin; venereal disease was denied. At 55, a week before 
admission, Hughes had a spell of unconsciousness for several 
hours, after which his speech was thick, and restlessness, in- 
somnia, and a wandering tendency set in. Visual halluci- 
nations, fabulation, tremors, " excited-looking " eyes, are 
described. He would sweep things from the dining-room 
table, pulled a hot stove Into the middle of the floor, at- 
tempted to sweep paint off the floor, and cut up a carpet 
with a knife. 

The patient on commitment November 5, 1904, was well 
developed and nourished. The mucous membranes were 
rather pale. Bruises and excoriations of limbs. Harsh 
breathing at the base of each lung. Enlargement of heart; 
sounds irregular. Accentuation of aortic second sound; 
tension fair, rate 80. Slight brachial arteriosclerosis. Abdo- 
men slightly distended. The urine contained a faint trace 
of albumin and many hyaline casts. 



PUZZLES AND ERRORS l8l 

Moderate tremor of extended hands. Slight tongue 
tremor. Romberg's sign absent (slight swaying). Con- 
siderable ataxia of extremities (inability to stand with foot 
on opposite knee). Vision poor. Hearing could not be 
tested accurately. Prompt pupil reactions with direct light. 
Slight consensual reaction in left pupil, absent in right. Deep 
reflexes equal and lively. 

Quiet and orderly at first. Later restless and noisy. 
Questions were answered at times relevantly, more often 
irrelevantly. Patient irritable, intractable. Required re- 
peated urging to take nourishment. Consciousness clouded. 
Orientation imperfect. Attendants are possibly " officers." 
Date September, 1995. Slight errors in repeating alphabet. 
Mistakes in Lord's Prayer with rhyming tendency. Simple 
arithmetical tests answered automatically with many mis- 
takes. More complex combinations incorrect. Handwriting 
tremulous (noted as " typical of general paresis "). Auditory 
hallucinations (answering invisible persons), "All right, I'm 
coming." Amnesia and confabulation. Q. " Have you 
had breakfast? " A. " No," (later) " Yes, I had a very light 
breakfast." Q. " What did you have? " A. " Anything 
that came along. A few green peas and beans that were left, 
bread and butter and pie. I had a good breakfast. Guess 
feed is very high." Q. " Give names of your sisters and 
brothers." A. " There are three or four I never see. I will 
have to think them up." (Later) — " Lillie, Abbie, Julia, 
George." On repetition of question, " Elizabeth, Julia, 
Annie and Lizzie." 

Delusions somewhat doubtful. At no time euphoria. 

The patient remained only nine days in the hospital, 
developing diarrhea a week after admission. 

Post Mortem Findings. The cause of death was bilateral 
bronchopneumonia of hypostatic distribution, accompanied 
by bronchitis and acute splenitis. The intestinal tract was 
normal (despite the diarrhea). No cultures. The heart 
showed acute myocarditis. 

The vessels in general showed no sclerosis, except that the 
aorta showed a few patches with calcification near bifurcation. 
There was a moderate degree of mitral sclerosis. The kidneys 



1 82 PUZZLES AND ERRORS 

showed a moderate degree of chronic interstitial nephritis. 
The heart weighed 530 grams and there was moderate dilata- 
tion of all the valves. 

There were some evidences of chronic disease outside the 
nervous system, namely, an obliterative pleuritis on the right 
side, chronic perisplenitis, and chronic external adhesive 
pachymeningitis. 

The nervous system showed a pia mater thin and trans- 
parent, with a moderate congestion of larger and smaller 
vessels. No noteworthy change of the brain substance or of 
the ventricles was found, except that the cerebral substance 
was of unusual firmness (autopsy twelve hours after death). 

It is clear that the brain was not wholly normal, exhibiting 
a general induration due in part to subpial gliosis and_in part 
doubtless to perivascular gliosis. Microscopically the tissues 
showed features of great interest, especially multiple focal 
neuroglia cell proliferations of a perivascular distribution, 
considerable subpial fibrillar gliosis of an unusually focal 
type, and a rather general subpial cellular gliosis. Histologi- 
cally, it seemed that this chronic progressive process had 
started, not so much in relation with dying nerve cells, as in 
relation with blood vessels. The perivascular deposits of 
neuroglia cells were confined almost exclusively to the in- 
fragranular cortex layers. It seems plain that the diagnosis of 
general paresis was not justified. It is probable that the diag- 
nosis neurosyphilis is not justified. The explanation may 
be that now and then cases of cerebral sclerosis may clini- 
cally imitate the neurosyphilitic process. It must be borne 
in mind that the diagnosis in this case was made, like the other 
cases at head of Part III, without the advantage of mod- 
ern systematic methods. Clinically speaking, of course, 
there was no definite Argyll- Robertson pupil, although the 
consensual reaction, slight on the left side, was absent in the 
right pupil. The general picture appeared to be one of 
the so-called demented form of paretic neurosyphilis. 



PUZZLES AND ERRORS 1 83 



Differential diagnosis between NEUROSYPHILIS 
and NEURASTHENIA. 



Case 45. Albert Robinson, a man of 28 years, was ship- 
wrecked on one of the Great Lakes. The ship was on the 
rocks for eight days, and Robinson was under a great strain. 
Ever after the wreck, Robinson had felt severe pain in the 
head, neck, and back, and a feeling of great weakness when- 
ever he exerted himself physically or mentally, and seven 
months after the wreck, he had several attacks of fainting. 

For a number of weeks he had worried a good deal about 
his inability to make money, especially as money was badly 
needed on account of his wife's approaching confinement. 
A few days before entrance, Robinson had become very 
forgetful, and was unable to recall, the night before entrance, 
where he had been during the day. On the whole, however, 
on mental examination no actual evidence of memory defect 
could be shown to exist. 

Physically, Robinson was entirely negative, except for 
some hard glands in each groin. Mentally, there was little 
to show except depression, worry over his financial condi- 
tion, and his inability to work. The serum W. R. proved 
negative. 

Diagnosis: On the whole, the diagnosis of psychoneu- 
rosis (see case Harrison (9)) due to the shock at the time of 
the shipwreck seemed to be proper. To be sure, the patient 
gave a history of a chancre at 25, treated for two years, after 
which he was declared cured. 

However, following up the clue of admitted syphilis, 
rigorous questioning elicited the fact that a few months 
before there had been diplopia, lasting part of a day. 

Lumbar puncture seemed desirable. The fluid was clear 
but contained 125 cells per cmm. with appropriately in- 
creased amounts of albumin and globulin. The spinal fluid 
W. R. was positive. The diagnosis of Cerebrospinal 
Syphilis seemed established. 



184 PUZZLES AND ERRORS 

The lesson of this case appears to be that perhaps we should 
never exclude syphilis until we have made an examination 
of the cerebrospinal fluid. The W. R. of the blood in meningo- 
vascular (non-paretic syphilis) is negative in many cases 
(the figure is sometimes set as high as 40%). 

Treatment: After a half dozen injections of salvarsan, 
all symptoms disappeared and Robinson went back to work, 
claiming to be in a better condition than for some time past. 

I. How shall we explain such a symptom as the transient 
diplopia? This diplopia is probably an example of 
a neurorecidive, but it will be observed that it occurred 
without salvarsan therapy. See discussion above under 
the case of Bennett (34), where the general result of 
the neurorecidive inquiry launched by Ehrlich early 
in the history of salvarsan therapy showed that pre- 
cisely similar phenomena had always occurred in 
neurosyphilis, whether under treatment or not. The 
anatomical and histopathological explanation of such 
phenomena is, of course, doubtful, but a review of the 
findings in the case of Alice Morton (i) will show how 
many apparently serious symptoms in neurosyphilitics 
are actually irritative or at least due to lesions which 
are entirely recoverable. We may suppose, first, a 
local proliferation of spirochetes; second, a local over- 
formation of toxic substances, directly or indirectly the 
product of spirochetosis; thirdly, a local exudation; 
fourthly, a local proliferation; fifthly, a combination 
of these phenomena, any or all of which may be regarded 
as but transient. We have sometimes found at autopsy 
very little exudate except in small areas; sometimes 
not more than a few mm. or cm. in superficial extent. 
Note, for example, the small areas of lymphocytosis 
demonstrable in but two foci in the case of Alice 
Morton, the paradigm placed at the beginning of this 
book. 



PUZZLES AND ERRORS 1 85 



NEUROSYPHILIS(?) in the SECONDARY STAGE 
of syphilis. HYSTERICAL symptoms. Diagnosis? 



Case 46. Alice Caperson was a colored girl of i8 years. 
She had acquired syphilis five months before admission to 
the hospital, and the secondary symptoms of this syphilis 
had just disappeared before admission. 

Very shortly after acquiring syphilis, the young negress 
began to act peculiarly. She describes herself as having 
a sort of nightmare, both when asleep and also when awake. 
For instance, she saw her dead grandmother. It appeared 
at first like a seraph ; then it came nearer to her and seemed 
to fill out ; and then was dressed precisely as her grandmother 
had been. This seraph appeared as though trying to tell her 
something, but she could not make out what the something 
was. The vision had appeared on two or three occasions. 

Our examination detected little beyond instability and 
irrltabiHty of mood with some depression. The patient 
readily fell to weeping. She soon made friends in the wards, 
however, and got on well. Physical examination was en- 
tirely negative but the W. R. of the blood serum was positive. 
The W. R. of the spinal fluid was negative, as was the gold 
sol reaction; there was an excess of albumin and a positive 
globulin test; there were seven cells per cmm. 

The psychiatric diagnosis of a case like that of Alice Caper- 
son would waver between hysteria and dementia prsecox. 
However, as for dementia prsecox there are hardly any 
typical symptoms. There Is Insight Into the hallucinations, 
which are hypnagogic. There are, however, no hysterical 
stigmata. 

The spinal fluid reaction is typical of the secondary stage 
of syphilis. It Is commonly said that in every case of syph- 
ilis the nervous system Is Involved at some period, if only 
to the degree shown In the present case. However, such 
involvement tends to disappear both with and without anti- 
syphilitic treatment, just as do the secondary skin symptoms. 



I86 PUZZLES AND ERRORS 

So far as syphilis is concerned, the prognosis under radical 
treatment is as good as usual. We are inclined to regard 
the case as one of the Hysterical or Psychopathic group 
and inasmuch as cases occurring in the developmental stage 
of a patient's life are of fairly good general prognosis, we 
are inclined to regard the prognosis in this particular case as 
good under proper therapy and hygiene. 

1. What is the relation of neuroses to syphilis? Neuras- 

thenia, chorea, hysteria, and epilepsy are often grouped 
(for example, by Nonne) as neuroses bearing at times 
important relations to neurosyphilis. (For the relations 
of neurasthenia, chorea, and epilepsy, see cases of 
Greeley Harrison (9), Margaret Green (72), and David 
Borofski (49), respectively.) As for the hysteria shown 
in Caperson, Charcot enumerated syphilis among agents 
provocateurs of hysteria along with alcohol, lead, ar- 
senic, and the like. Fournier has also considered the 
problem. It is clearly necessary to show that before 
infection there were no hysterical symptoms, and that 
the hysteria developed during the operation of the syph- 
ilitic process, and it is probably necessary to show that 
the symptoms will clear up under antisyphilitic treat- 
ment, if we are to concede the existence of a syphilitic 
hysteria. 

2. What are the evidences of neurosyphilis in the secondary 

and primary stages of syphilis? As above stated, the 
findings in Caperson are typical enough. Wile and 
Stokes at first stated that 60 to 70% of the secondary 
syphilitics show changes in the spinal fluid; in a fur- 
ther article they maintain that probably every case 
shows such changes and that clinical symptoms of 
neurosyphilis of the secondary period can probably be 
determined. They claim that it is probable also that 
the same holds for primary syphilis itself. The im- 
portance of these claims lodges partly in the relation 
of these early signs of neurosyphilis to the whole ques- 
tion of latency and to the question of paresis sine paresi. 
For a discussion of paresis sine paresi see cases Lawlor 
(25), Vogel (52). 



PUZZLES AND ERRORS 1 87 



Differential diagnosis between NEUROSYPHILIS 
and MANIC-DEPRESSIVE PSYCHOSIS.* 



Case 47. As in other instances (compare Martha Bartlett 
(21) and Annie Monks (85)) so also in the case of Ethel Hunter, 
a woman 6i years of age, there was no initial suspicion of 
neurosyphilis. Mrs. Hunter was brought to the hospital 
stuporous as a result of an overdose of paraldehyd. The 
paraldehyd had been administered by a physician to combat 
insomnia and agitation. As soon as Mrs. H. had recovered 
from the drug stupor, this agitation appeared once more, and 
it was clear that she was suffering from marked depression. 
There was tremendous worry over the sickness of a woman 
with whom the patient lived. The patient was very self- 
accusatory, blaming herself for many things that had hap- 
pened in the household. Besides her agitation, depression, 
self-accusations, and insomnia, the patient showed a good 
deal of the symptom frequently termed " retardation " — 
a kind of lagging of all mental processes found, according to 
Kraepelin, In manic-depressive psychosis. 

Accordingly, the diagnosis of manic-depressive psychosis 
might well have been rendered. The fact that the psychosis 
so far as known began In the involution period was not against 
the diagnosis since the so-called involution-melancholia of 
this period is at least in a certain fraction of cases nothing 
more or less than a form of manic-depressive psychosis. 
However, the physical examination made the diagnosis of 
manic-depressive psychosis a little doubtful. There was a 
superficial thickening of the arteries (blood pressure : systolic, 
170; diastolic, 104), which thickening would not in itself be 
against the diagnosis of manic-depressive psychosis. (In 
point of fact, arteriosclerosis is rather common late in this 

* A. M. Barrett has recently discussed this subject In a 
paper In the Journal of the American Medical Association, 
Vol. LXVn, Dec. 2, 1916. 



1 88 PUZZLES AND ERRORS 

disease and previous attacks could not be excluded on the 
basis of available history.) The contracted pupils were 
irregular and both reacted sluggishly to light, although better 
to accommodation; the right pupil was larger than the left. 
The arm reflexes were pretty active. The left knee-jerk 
could not be obtained, nor was the right knee-jerk more than 
very sluggish. The Achilles reflexes could not be obtained. 
Although there was not a positive Romberg sign, there was 
a considerable swaying in Romberg position. There was 
no speech defect. The other reflexes showed nothing ab- 
normal. On the whole, we had to conclude that, although 
Mrs. Hunter might be an instance of manic-depressive psy- 
chosis, still there was much of neurological interest in the 
case. 

This conclusion was emphasized when the W. R. of the 
blood serum was found to be positive. The spinal fluid W. R. 
was also positive, and the gold sol index was of the " paretic " 
type. There were 74 cells to the cmm. Globulin stood at 
+;+++, and albumin at + +++. 

This case, therefore, again illustrates, as well the protean 
nature of General Paresis (the diagnosis rendered), as the 
doubtful value of making a psychiatric diagnosis without 
due consideration of the physical examination and laboratory 
findings. How easy might it have been, at least some years 
ago, to consider that this patient of 61 years had suffered a 
slight shock at some previous time (left knee-jerk absent), 
but was as a matter of fact a case of manic-depressive 
psychosis with a vascular complication ! 

Note: We must again duly insist that the merely sluggish 
light reactions of the pupils in such a case as this do not 
especially point to general paresis. The literature seems to 
establish that sluggishness of light reaction precedes the 
classical Argyll-Robertson pupil. Yet it does not do to say 
that, if the Argyll-Robertson pupil pretty conclusively points 
to neurosyphilis (for exceptions see cases Falvey (55), Murphy 
(60)), then a sluggish pupillary reaction to light looks in the 
same direction. Sluggishness may precede stiffness in many, 
or perhaps all, cases, but sluggishness of pupils is a frequent 
phenomenon outside the syphilitic group of cases. 



PUZZLES AND ERRORS 1 89 

1. What part is played by emotional shock and psychic 

causes in the starting up of general paresis? The 
answer to this question cannot be definite. That a 
paretic process can be started up after trauma is ad- 
mitted on all sides; but we here suppose actual physi- 
cal or chemical brain disturbance permitting increased 
spirochetosis or inflammatory reaction. In the case of 
psychic shock, or what might be called psychogenic 
general paresis, our best resort will be to the indirect 
effects of hormone action, or of vasomotor and other 
autonomic disturbances produced directly or indirectly 
by emotion. We are clearly here dealing with material 
too speculative to be of practical service at this time. 

2. Was the depressive drug therapy in the case of Hunter 

justifiable? The paraldehyd had been administered by 
a physician apparently on purely symptomatic grounds 
to combat the insomnia and agitation of this woman of 
61 years. With all due acknowledgment of the dlfft- 
culties of private practice, we must insist that when 
ordinary measures in the relief of insomnia and agitation 
are insufhcient to curb these conditions, then a positive 
danger ensues with the larger doses. As a rule, with 
these larger doses and with the withdrawal of sen- 
sory stimulation, the patients relapse into a stupor of 
grave moment. We need only recall the situation in 
delirium tremens where adequately depressive drugs 
often tend to kill the patient. 



190 PUZZLES AND ERRORS 



Case for diagnosis. Errors in the diagnosis of 
NEUROSYPHILIS are possible even when abun- 
dant clinical and laboratory data are available. 



Case 48. The first error chosen for demonstration is that 
in the case of the machinist, Milton Safsky. 

Safsky, about 8 months before his entrance to the hospital 
in the 42d year of his life, had begun to lose strength, to grow 
thin and pale, and to suffer from an extreme and continuous 
thirst. He was said to have drunk as much as 6| gal. in a day, 
and passed appropriately large quantities of urine. After a 
time, his management at a general hospital became difficult, 
as Safsky became confused, cried " hysterically," and was at 
times very noisy. He sustained a marked memory loss, 
seemed to show visual hallucinations, and complained of 
headache, both frontal and occipital, and of pain about the 
eyes. Sometimes the patient was very euphoric and ex- 
pressed what seemed to be delusions of grandeur, saying he 
was wealthy and Qwned. many machine shops. 

Some symptoms, e.g., polydipsia and polyuria amounting 
to a diabetes Insipidus, associated with headache and arrested 
attention, suggested possibly a new growth in the pituitary 
region. The mental symptoms might naturally be supposed 
to be due to some Infiltration or pressure effect of Intracranial 
growth. After admission to the Psychopathic Hospital, the 
patient was found difficult to arouse, although he could 
eventually be aroused. His orientation proved to be as poor 
as his memory. From time to time, the patient became a 
bit more intelligent and able to execute requests. 

The physical examination was in general almost entirely 
negative. Neurologically, the pupils were markedly con- 
tracted and reacted slowly to light, though they were other- 
wise normal. The deep reflexes were all somewhat lively, 
though equal. The umbilical and cremasteric reflexes in 
particular were present. Systematic examination revealed 
no other reflex disorder, nor any disturbance of sensation. 



PUZZLES AND ERRORS I9I 

There was a coarse tremor of the extended hands. There 
were no phenomena of importance in the visual fields. 

As against the diagnosis of growth, pituitary or extra- 
pituitary (diabetes insipidus and headache), a hypothesis of 
neurosyphilis had to be considered. Not only were the con- 
tracted, slowly-reacting pupils and the active deep reflexes 
suggestive, but the euphoria with grandiose ideas looked 
entirely consistent. As for the polyuria, one had to think of 
the so-called syphilitic polyuria of the textbooks, which is 
regarded as a more or less characteristic result of syphilitic 
involvement of the basis cerebri. Moreover, the W. R. in 
the spinal fluid proved to be slightly positive; 146 cells per 
cmm. were found therein; there was a large quantity of 
globulin, and a very marked increase in albumin. These 
observations seemed to be exceedingly suggestive of a cerebral 
syphilis. 

However, as the case progressed, the diagnostic situation' 
changed. The W. R. upon a second puncture fluid proved 
negative. After some weeks, characteristic symptoms of in- 
tracranial pressure developed; the diagnosis of Brain Tumor 
had to be taken as established, and there is no doubt of its 
correctness. 

1. What is the explanation of the weakly positive W. R. in 

Saf sky's spinal fluid? An explanation is not easy to 
find. Possibly we may regard the reaction as an ex- 
ample of error in technique. It is even possible that it 
may have been produced by exudative products in the 
spinal fluid. 

2. What precautions may be taken against an error in 

diagnosis such as was first made through the positive 
spinal fluid Wassermann in the case of Saf sky? First, 
repetition of the W. R. ; secondly, it is very unusual 
to find a weakly positive W. R. in a case with such 
marked excess of albumin and such very marked in- 
crease of globulin as was shown by this case. 

3. How can we explain the inflammatory products in the 

puncture fluid? Superficial brain tumors are fre- 
quently associated with a so-called meningitis sym- 
pathica. The products of such meningitis are ex- 
hibited: viz., globulin, albumin, and pleocytosis, exactly 
as shown in Saf sky. 



192 PUZZLES AND ERRORS 



Can PARETIC NEUROSYPHILIS ("general 
paresis ") appear clinically EARLY (e.g., two years) 
after the initial syphilitic infection? 



Case 49. David Borofski, a street car conductor, 27 years 
of age, suddenly had a convulsion while at work in his car. 
For four months Borofski continued to have rather numerous 
convulsions, was finally compelled to discontinue work, and 
resorted to the Psychopathic Hospital. It appears from his 
own story that, about two years before, he had had a chancre, 
for which he had been treated at a general hospital syphilis 
clinic, and of which he was told he was cured. With a pro- 
gressive loss of memory and with convulsions, Borofski be- 
came much concerned about himself, and was finally per- 
suaded by his fellow-workers to come to the Psychopathic 
Hospital. 

The convulsions were described as follows: The patient 
gives a short cry, has convulsive movements for about ten 
minutes, remains unconscious for perhaps half an hour, and 
wakes with headache, dizziness, and a feverish appearance. 
Sometimes the attacks were more severe, with frothing at 
the mouth, biting of lips, and loss of sphincter control. There 
were also slight attacks, occurring almost every day, without 
loss of consciousness; these latter attacks consisted of diz- 
ziness, inability to speak for a few seconds, and some arm 
twitching. 

Physically, Borofski was well developed and nourished, with 
a blood pressure of 160. The only abnormal phenomena 
neurologically were absent knee-jerks and ankle- jerks, slug- 
gish pupillary reactions, and slight tremor of the hands. 

Mentally, despite suggestive complaint of amnesia, the 
memory was found to be fairly good but knowledge of cur- 
rent events and school knowledge was poor. The simplest 
problems in arithmetic Borofski gave up. 

The first diagnosis in such a case would naturally be 
epilepsy. However, when an epileptic or epileptiform at- 



PUZZLES AND ERRORS 1 93 

tack occurs for the first time in adult life, the chances are 
probably against an idiopathic epilepsy. (This is not a 
universal rule but will serve.) Borofski himself, moreover, 
gave a history of syphilis. And the very nature of the attacks, 
with arm twitching and without loss of consciousness, would 
not readily fit Into the frame of the idiopathic group. The 
absence of certain reflexes and the sluggish pupils are naturally 
also suggestive of syphilis, although not convincing. 

The W. R. of the serum proved positive, as did that of 
the spinal fluid. The gold sol reaction was characteristically 
"paretic"; there was an excess of albumin and a positive 
globulin, and there were 15 cells per cmm. There could be 
little or no doubt of the diagnosis of some form of neuro- 
syphilis. The laboratory picture was consistent either with 
general paresis or with cerebrospinal syphilis. So far as we 
are aware In the present stage of knowledge, the two conditions 
can hardly be differentiated unless we choose to rely on 
therapeutics. However, it is exceedingly rare for general 
paresis to occur only two years after the original infection. 
If we can trust this statistical fact, we shall perhaps be wiser 
to term the case of Borofski one of Diffuse Cerebrospinal 
Syphilis, and not one of paresis. 

Treatment: Borofski was put on antisyphilitic treat- 
ment consisting of 0.6 gram of salvarsan twice a week and 
potassium iodid, together with intramuscular injections of 
mercury salicylate. The convulsions then ceased. After 
four months Borofski returned to work, and he has remained 
at work for a year. He has never regained his former health. 

Fifteen months after beginning of treatment the laboratory 
tests were again made (there had been more than 60 injec- 
tions of salvarsan), and the cell count and gold sol reactions 
were found to be negative. Globulin and albumin were 
also in smaller amounts than in the original examination. 
However, the W. R. of the serum and the spinal fluid remained 
positive. 

Head and Fearnsides state that cases of cerebrospinal 
syphilis should return negative spinal fluid tests after six 
months of treatment. Upon this criterion of Head and 
Fearnsides, Borofski would not be a case of cerebrospinal 



194 PUZZLES AND ERRORS 

syphilis; but it is probably impossible to separate various 
forms of neurosyphilis into categories on any such grounds. 



1. Shall case David Borofski be regarded as one of paretic 

neurosyphilis ("general paresis") ? He has returned to 
work and has remained at work, though without regain- 
ing his former health. In any event, however, he does 
not offer the typical picture of inevitable decline and 
death presented by the typical case of Pietro Martiro 
(15) presented in our discussion of systematic diag- 
nosis. However, we could not upon laboratory grounds, 
or even upon the ground of clinical observation, dis- 
tinguish Borofski from Martiro; Borofski has greatly 
improved; Martiro is dead. Borofski developed his 
obvious neurosyphilis only two years after the original 
infection. The conservative syphilographer might, ac- 
cordingly, reply that David Borofski is not a typical 
case of paretic neuros3/philis ("general paresis") either 
in the length of the incubation period for his neuro- 
syphilitic symptoms, or in his outcome. 

2. What is the cause of such convulsions as those developed 

by David Borofski? Evidence from clear cases of 
general paresis with convulsions leads to the hypothesis 
that such convulsions as those developed by Borofski 
are not necessarily based upon frank destructive 
lesions such as would be produced by the plugging of 
terminal arteries. They may well be produced through 
the activities of minor lesions, only demonstrable by 
microscopic methods, either through properly disposed 
cell losses or by the pressure of exudate, or even by 
endotoxins or other substances derived from the bodies 
of dead or living spirochetes. 

3. Aside from the well-known syphilitic epilepsy due to 

meningitis, is there a non-meningitic epilepsy (such 
a disease as Fournler formerly described under the term 
parasyphllltic epilepsy)? We dismiss from discussion 
the so-called symptomatic epilepsies which are the 
result of a gross organic disease of the brain substance 
or its membranes, and which do not differ so far as we 
are aware from organic epilepsy produced by other 
gross lesions of an identical size and structure. These 
symptomatic epilepsies may be partial, or even may 
present the appearance of generalized epilepsy. We 
may also leave out of account those epileptic pictures 
which are produced in general paresis itself, and which 



PUZZLES AND ERRORS 1 95 

may be viewed as nothing but partial phenomena of 
general paresis. The kind of so-called " parasyphilitic " 
epilepsy that Fournier described is a kind of epilepsy 
that cannot be distinguished from genuine epilepsy, in 
which the sole disease-phenomenon throughout a long 
period of time consists of epileptic convulsions. It ap- 
pears that these " parasyphilitic " imitations of genuine 
epilepsy occur in individuals with a very long post- 
infective " incubation period," but that there are some 
cases in which the epilepsy appears, on the contrary, 
in the very earliest stages of syphilis. The attacks 
are a little less common than those of idiopathic epi- 
lepsy; they have the same apparently causeless be- 
ginning; are associated with complete amnesia; and 
are followed by characteristic dazed states. The 
patient's intelligence, however, suffers little. Now and 
then a case reacts well to antisyphilitic treatment ener- 
getically pushed. (Spontaneous long remissions in 
non-syphilitic epilepsy must be remembered.) Petit 
mal attacks occur sometimes between the more severe 
attacks. In short, it would appear that there is a 
group of syphilitic epilepsies in which the brain shows 
no gross structural lesions, which accordingly do not 
exhibit any Jacksonian appearances, and which last 
a comparatively long time without changing their 
character, and often without being especially altered, 
for the better by any form of antisyphilitic treatment. 
This condition is sometimes known as a post-syphilitic 
epileptic neurosis. Nonne had been able to collect up 
to 1902 some 12 cases from his own service. 

4. Would it be proper to call Borofski a case of taboparesis? 

Absent knee-jerks in a victim of paretic neurosyphilis 
should not be used to suggest a diagnosis of taboparesis. 
This question of terminology has been discussed above, 
under Sullivan (i6). 

5. What is the mechanism by which the amnesia of a case 

like Borofski is produced? The answer runs in the 
same termxS as the answer to the questions concerning 
the cause of convulsions. The amnesia in general 
paresis has surprising functionality. A study of autop- 
sied cases of general paresis has shown that amnesia 
is practically as common in cases without marked 
destruction of brain tissue as in cases with atrophy of 
classical extent and depth. The clinical recovery in 
this case was practically complete in respect to memory. 
We must regard the amnesia as not due to the destruc- 



196 PUZZLES AND ERRORS 

tion of storage cells bearing the so-called neurograms 
(Morton Prince). 

6. What is the explanation of the persistently positive W. 

R.'s of the serum and spinal fluid associated with 
diminished globulin and albumin tests, a negative gold 
sol reaction, and normal cell count ?_ See discussion 
under Case Martha Bartlett (21). 

7. How atypical is the early development of paretic symp- 

toms in David Borofski? C. B. Craig has collected, 
in 100 cases of brain syphilis (a list including both 
paretic and non-paretic cases), some data on this 
point. The shortest period reported by Craig was in 
a case in which the neurosyphilitic symptoms appeared 
one month after infection. Craig found three cases 
where symptoms appeared in six months, and six cases 
within a year. The longest post-infective period of 
Craig's list was thirty years. Our case of Chatterton 
(73) developed symptoms 33 years after infection and 
Washington (66), forty years after infection. Nonne 
casts some doubt on statements to the effect that 
tabetic symptoms may occur three to four months 
after infection. It seems to be admitted that pupillary 
anomalies and reflex changes may occur in the early 
secondaries and may recover under antisyphilitic treat- 
ment. Nonne' s case of longest post-infective interval, 
like that of Craig, was one of 30 years. 

Myerson has reported a 20-year old patient who 
acquired chancre April i, 191 1 (spirochetes demon- 
strated) ; salvarsan was administered April 20th. There 
were no secondary symptoms, but in May, headache, 
visual disturbance, vertigo, and other symptoms de- 
veloped (neurorecidive) . Upon June 20th, that is, 
II weeks after development of the chancre, aphasia 
and astasia developed, with numbness of the left side. 
At this time, the pupils were slightly irregular and un- 
equal but reacted normally. The signs in the fluid 
were positive. Upon this question see our cases of 
Bright (121) and Bennett (34). 



PUZZLES AND ERRORS 1 97 



HEMITREMOR following hemiplegia in PARETIC 
NEUROSYPHILIS ( * * general paresis ") . Autopsy. 



Case 50. Achilles Akropovlos, 39 years, had symptoms 
six months before commitment to Danvers Hospital. There 
were attacks of confusion, difficulty in walking, and speech 
defect, resulting in an entire incapacity to work and 
eventual commitment. Rather unusual and striking was a 
very marked tremor, apparently limited to the right side of 
the body. Physically, Akropovlos was normal, but neuro- 
logically he showed, in addition to the marked right-sided 
tremor, a marked speech defect, and a degree of ataxia. 
The tendon reflexes were very active, but there were no ab- 
normal reflexes, and the pupils reacted normally. According 
to the history, the difficulty had followed a slight attack of 
apoplexy. Mentally, there was a marked confusion. The 
blood serum and the spinal fluid were both positive to the W. 
R. ; globulin was present, and albumin was increased; there 
were 43 cells per cmm. There was hardly any diagnosis to 
make except general paresis. 

Death followed 18 months later, or two years after onset 
of symptoms. Increasing weakness, emaciation, and de- 
mentia preceded death. Autopsy confirmed the diagnosis of 
Paretic Neurosyphilis. 

I. What is the usual cause of death in general paresis? 
Intercurrent disease very frequently occurs in general 
paresis, and such intercurrent disease is then given as 
the cause of death. As a matter of fact, however, one 
feels that in many of these cases the intercurrent 
pneumonia or infection — frequently of the bladder, 
— bedsores, sepsis, and the like, are merely accidental 
incidents in a condition that is leading to death, and 
which has caused a lowered resistance to infection. 
In certain instances where nursing is exceptionally good 
and where no such infection occurs, the patient con- 
tinues to grow weaker and weaker, paralyses of all the 
muscles follow and finally paralysis of deglutition or 



198 PUZZLES AND ERRORS 

respiration may lead to death. The emaciation and 
paralyses may be of such a grade that the patient is 
entirely devoid of fat and unable to move at all. Not 
infrequently vascular crises occur, and one of these may 
be responsible for death. 
-2. What was the cause of the hemitremor? The hemi- 
tremor suggested an irritative or destructive lesion in 
the motor path. Delving into the history it was 
learned that the patient had had a shock followed by 
a right hemiparesis. This had cleared up leaving the 
tremor as a residuum. The autopsy disclosed a reddish- 
brown pigmentation and fibrous thickening of the pia 
over the left motor area, confirming the idea of a 
previous hemorrhage. As a rule the shock phenomena 
occurring in paresis clear up more completely and no 
gross lesion is visible post-mortem. However, cerebral 
hemorrhage must be expected in any person suffering 
from syphilis, and is no rarity in paretic neurosyphilis. 



PUZZLES AND ERRORS 1 99 



PARETIC NEUROSYPHILIS (*' general paresis ") 
with NORMALLY REACTING PUPILS. History 
of trauma. Autopsy. 



Case 51. Daniel Wheelwright, a barber of EngUsh ex- 
traction, 57 years of age, had had a sunstroke at 15. At 42, 
there had been pneumonia, after which an attack of rheuma- 
tism was said to have kept the patient from work for a year. 
There was trauma of head (falHng wrench) at 44. This blow 
on the head was the assigned cause of the mental disease, 
symptoms of which, however, did not develop until about the 
first of September, 1905, about three months before entrance, 
January 9, 1906, and about six months before death, March 
20, 1906. 

It seems that the patient had begun to change in manner; 
he had become despondent and apathetic, silent, and som- 
nolent. Two weeks later, he stopped working, began to 
read the papers once more, and became somewhat more 
cheerful. 

About Thanksgiving, Wheelwright got up at midnight, and 
remained up, lighting all the fires and talking continuously. 
During the next two weeks, he talked much to himself, 
laughing out at times. About two weeks before Christmas 
he went out and started to make a sidewalk of old boards, 
working in his shirtsleeves, without a hat. He would work 
until midnight making screens for windows. During the 
day, he would go out and give money to passing children; 
would offer to pay the grocer twice as much as articles were 
worth. 

On the day before Christmas, he put out all the fires and 
lights in the house, sent all the family to bed, and opened all the 
doors. Christmas morning, he rose early and got the washtubs 
ready. He helped his compliant wife to do the washing, then 
put out all the fires and opened the windows. After Christmas, 
he began to tell how rich he was going to be through starting 
a garden and by making butter. He bought six or seven 



200 PUZZLES AND ERRORS 

quarts of milk daily, and procured carrots and oranges, 
grinding them up to color the milk. January 9th he was 
committed to Danvers Hospital. 

Physically, there were few symptoms. Neurologically, there 
was a tremor of tongue, fingers, and face. The knee-jerks 
were lively. The pupils reacted normally; the patient was 
restless, pacing up and down. There was a speech defect 
demonstrable with test phrases. Orientation was imperfect 
for time and for place. Hand-writing was poor, memory 
impairment was marked, but the patient was given to fabri- 
cation as to past events. A characteristic sample of state- 
ments : 

" Do you know that this is an insane hospital?" "Yes; 
there are two or three men here out of their heads. I could 
cure them with my hands but they won't let me. I could get 
all the sick men on their feet just by rubbing them. I can 
do anything with my hands. I can build a house by just 
sitting down and thinking about it. I can whip all the men 
in this place. I have better sense now than I ever had in 
my life." 

Again, " How long have you been here?" " Over three 
months ; they have put me in heaven three times since I have 
been here. They killed me, crushed my heart, and turned 
my blood to water. I am all right now. I let the sun shine 
on my heart and it brought it together. I can whip every 
man in here as fast as they come up." 

Again, " I will make a million dollars on my garden when 
I get it. I can make a million dollars on half an acre. 
I can do anything. I can move this house by just thinking 
of it." 

During a special examination, the patient told how he had 
fastened wings on his hands and feet, and how he had gone to 
heaven; he told how he had soared high above the earth, and 
how differently the stars look when up near heaven than they 
do from the earth. He spoke of seeing angels and of the 
beauties of heaven. 

The diagnosis of Paretic Neurosyphilis was confirmed 
at autopsy. 



PUZZLES AND ERRORS 201 

What is the significance of the normally reacting pupils? 
While it is usual to find pupillary anomalies in 
neurosyphilis, these changes are not an essential part 
and it is not rare to find normal pupils in all forms of 
neurosyphilis. It is less frequent to find a normal 
pupil in tabetic than in diffuse or paretic neurosyphilis. 
In paretic neurosyphilis it is the rule to find pupillary 
changes during some stage of the disease, but not neces- 
sarily early. At times the pupillary sign may be one of 
the earliest signs of neurosyphilis — again it may occur 
only as a late symptom, if at all. One of the most im- 
portant of the pupillary signs is irregularity of contour. 
While this does not always mean neurosyphilis it is 
highly suggestive and certainly indicates careful exami- 
nation even though the W. R. in the blood be negative. 

What was the relation of trauma to the development 
of the neurosyphilitic symptoms? It is, of course, the 
rule in all forms of mental disease to have some factor 
offered by the patient or relatives as the cause of the 
psychosis. Often these assigned causes are minor 
events thought of only after the later appearance of 
symptoms. In this case it was not thought that the 
trauma had any causal effect. For a discussion of 
trauma and neurosyphilis see cases Joseph O'Hearn 
(90), Levi Sussman (91), and Joseph Larkin (92). 



202 PUZZLES AND ERRORS 



NEUROSYPHILIS, probably PARETIC, with 
symptoms highly suggestive of MANIC-DEPRES- 
SIVE PSYCHOSIS. 



Case 52. Bessie Vogel* was admitted to the Psycho- 
pathic hospital New Year's day, 191 5, in a very much ex- 
cited condition. The family history is very meagre, and all 
that is of significance is that mother has always been very 
" nervous." The records in part: 

Past History. Very healthy as a child, and except for oc- 
casional throat trouble and headache had no physical ailments 
until eight years ago, when she had an operation for appen- 
dicitis, and two and one-half years ago was operated upon for 
hernia and adhesions. Following this she began to show a 
lack of energy, neglected her housework, was much depressed, 
wept frequently, complained constantly of pain in various 
places, and was ill-tempered. In about five months she 
improved, and then after a couple of weeks at the shore 
seemed entirely well. 

Present Illness. In November, 1914, that is, about 
seventeen months after the recovery from the previous de- 
pression, she again began to show practically the same 
symptoms. She was depressed, could not sleep, and would 
get up in the night and sew; was self-centered and hyper- 
sensitive, then became restless and nervous; wanted to go 
shopping and out for dinner; went to New York and then to 
New Bedford. Symptoms became more marked; she be- 
came very ill-tempered, threatened her husband when angry 
over trifles, threatened suicide, then began to get active and 
spent money extravagantly. At the end of two months, that 
is, Jan. I, 191 5, she was admitted to the hospital. 

Physical Examination. A small, thin woman, appearing 
to be about 45 years old (actual age 37). Aside from the 

* Reprinted from an article by Southard & Solomon: 
" Latent neurosyphilis and the Question of Paresis sine 
paresis Boston Medical and Surgical Journal, XXIV, i. 



PUZZLES AND ERRORS 203 

absence of teeth and the operation scars, the general exam- 
ination is negative. Neuromuscular system: The pupils 
are round, regular, equal, and react to light and accom- 
modation, but do not hold very well. Extraocular move- 
ments well performed, no palsies of facial muscles, tongue 
protruded medially without tremor. Uvula is raised sym- 
metrically. Biceps and triceps and supinator reflexes are 
present and brisk. Patellar and Achilles reflexes are equal 
on the two sides and brisk. Abdominal skin reflexes not 
obtained. Plantar reflex active and flexor in type. No 
Babinski, Gordon, or Oppenhelm. No tremors. 

Wassermann reaction serum positive. Examination of 
spinal fluid: clear, globulin + + + +, albumin + + + + ; 
cells, 130 per cmm.; small lymphocytes, 79.9%; large lym- 
phocytes, 14.1%; polymorphonuclear leucocytes, 4.6%; 
plasma cells, 0.7%; endothelial cell, 0.7%. W. R. positive. 
Gold sol reaction, 55555522 + -. 

Mental Examination. On admission patient showed great 
psychomotor activity, was very playful, marked flight of 
ideas, was expansive, very emotional, very erotic. She 
slept very little, appetite was poor, and she lost weight 
rapidly. Orientation and memory intact. No hallucinations 
elicited. In about three weeks improvement began, and at 
the end of eight weeks she appeared practically recovered. 
On April 9, 1915, — that is, 13 weeks after admission, — 
she was allowed home on visit. On leaving, she appeared 
normal in every way. There was no evidence of psychotic 
symptoms, she had good insight, and physically there was 
absolutely nothing of a neurological nature that was abnormal. 
'^^This case, with the history of a previous depression and its 
clinical picture during the acute stage, and its recovery, 
is certainly in every respect typical of manic-depressive in- 
sanity, and only the positive result of the six tests causes us 
to put it in the group of General Paresis. Only the further 
course will shed any light as to the correct significance of 
these findings, and even then we shall not be too sure that we 
had not been dealing with a manic-depressive ps^^chosis in a 
latent neurosyphilitic. We would strongly emphasize the 
point that at the present time this patient presents no mental 



204 PUZZLES AND ERRORS 

or physical signs of cerebrospinal syphilis or general paresis; 
but the six tests are still positive. This case differs from the 
ordinary general paresis remission in that there is not a single 
physical sign of paresis present. 

There are many transitional cases between this case which 
shows no symptoms or signs of neurosyphilis except the lab- 
oratory tests, and the typical case of general paresis. Thus 
we have cases with slight character change and no physical 
signs except rare ** seizures." On the other hand, in many 
cases the presence of abnormal neurological phenomena with- 
out definite mental signs is first noted. Certain remitted 
cases show only some slight pupillary or reflex abnormality. 
We believe^we have here added the last link in the chain 
between the primary and quaternary symptoms. 

This case is illustrative of several which we have pub- 
lished elsewhere under the name of paresis sine paresi or 
latent neurosyphilis to illustrate how all the laboratory signs 
of neurosyphilis may be present in a patient without any 
physical or mental symptoms that may be correlated with 
these findings. 

We summarize our discussion of this as follows : 

1. There is a group of cases showing the laboratory signs 
characteristic of central nervous system syphilis : {a) positive 
W. R. in the serum, (5) positive W. R. in the spinal fluid, 
(c) pleocytosis, {d) excess of albumin, and (e) of globulin in 
the spinal fluid, (/) gold sol reaction of central nervous 
system syphilis, and which show no sign or symptom of 
neurosyphilis. 

2. We believe these cases represent a form of chronic 
cerebrospinal syphilis, probably paretic in type. 

3. They have the greatest theoretical and practical 
significance in the consideration of the life history of neural 
syphilis, in the concept of Allergie, in regard to results of 
treatment, and finally as to the evaluation of the laboratory 
tests. 

4. Here is perhaps offered the last link to form a complete 
chain between the symptoms of the primary stage of syphilis 
and its final termination of life as the result of the diseases 
cerebrospinal syphilis or general paresis. 



PUZZLES AND ERRORS 205 



SYPHILIS (?); EXOPHTHALMIC GOITRE; neu- 
rosyphilitic old lesion of optic thalamus ; unilateral 
induration and atrophy of left cerebral cortex. 
Autopsy. 



Case 53. Carrie Pearson, a housewife 25 years of age, died 
at Danvers Hospital less than a week after admission, and it 
was at first stated that her symptoms had lasted but two 
weeks before admission. In point of fact, a further investi- 
gation showed an important succession of symptoms, lasting 
some four years. 

Carrie had been considered a healthy child, going to school 
at the usual age, and progressing well with her studies. She 
however, left school in the ninth grammar grade, at the age 
of 15, and went to work in a milltown. She married a worth- 
less person at the age of 18, and lived with her husband 
for three years. There was one child born a year after 
marriage. Two years later, however, a tremendous goitre 
had developed such that her neck was described as " out 
square with the face," and at the same time the patient's 
eyes had become prominent. 

About two weeks before admission, she had gone to a 
neighboring town to take care of a sick woman, but during 
her endeavor to be a nurse, she had broken out into a mania, 
tearing up furniture and bedding, and talking irrelevantly 
for a period of four days. She also showed insomnia and 
continually tore off her clothing from her body. 

Upon examination, the marked enlargement of the thyroid 
gland together with the prominent eyeballs, husky voice, and 
pulse rate of 150 per minute, were entirely consistent with 
the diagnosis of exophthalmic goitre. The patient described 
herself as " Carrie Nation." Asked to write her name, 
she took the pen and tried to spatter ink, wrote hurriedly and 
carelessly her maiden name and several words without ap- 
parent meaning. Asked to write, " God save the Common- 
wealth of Massachusetts," she wrote: " God save the 



206 PUZZLES AND ERRORS 

common pal U S Spe Manor Gen, or til pat. Since Lord, or 
no prime in Hear to the God Tel. Ho. n and or Mabel, or gal." 
After this, she took paper and wrote meaningless scrawls, 
saying that it was Japanese writing. There was much 
motor restlessness with distractibility, pointing and gri- 
macing, mimicking the actions of those about her. 

Death occurred from exhaustion, and the case might not 
have been regarded as unusual except for the autopsy, which 
showed a peculiar brain lesion, described below. The point 
of greatest interest in the case was the fact that syphilis is, 
although not proved to exist by laboratory tests, beyond 
question a factor in the case. Although the woman had 
given birth to a normal child, who is still alive, yet in the 
period of a few years her breasts had atrophied, her hair had 
disappeared from the axilla and from the pubes; varicose 
veins had developed in both legs. Whereas there was little 
or no fat over the chest or back, the omentum and mesentery 
were very plentifully supplied with fat. It is probable, then, 
that we are dealing with a case of exophthalmic goitre some- 
how of syphilitic origin. The brain lesion is consistent with 
this hypothesis. 

Autopsy, March 3, 1907. ^ Four hours post-mortem. 

Body length, 165 cm. Body of a well developed and 
well nourished young woman. Lividity in dependent 
parts. Purplish discoloration of left thigh to knees. 
Skin rough and scaly. Petechial eruption over chest. 
Neck thick, protrudes anteriorly. Varicose veins over 
upper parts of calves on both legs. Eyes protruding, 
not covered entirely by lids. Pupils equal, dilated. 
Subcutaneous fat very deep over lower part of body. 
Very little fat over chest and back. Breasts are very 
small, apparently atrophied. Normal amount of hair 
on head, slight amount over pubes. Axillary hair ab- 
sent. Fat on section of a light yellow color. Omentum 
extends to pubes, plentifully supplied with fat. Large 
amount of mesenteric fat. Appendix normal. In- 
testines smooth and glistening. Slightly injected. No 
fluid in peritoneum. Uterus small, retro verted. 

Head: Hair in good quantity. Scalp normal. 
Calvarium shows diploe. Dura Mater over left 
cerebral hemisphere inseparably adherent to calvarium, 



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A B 

Cortical hemiatrophy — A, relatively normal right precentral ( "motor" ) cortex; B, 
atrophic left precentral. 
Note in B: 

1. Absence of giant pyramids of Betz (corticospinal, upper motor neurones). 

2. Superficial (subpial) condensation of tissues with sclerosis (gliosis). The tissues 
in all areas examined on the left side yielded this effect. 



PUZZLES AND ERRORS 20/ 

over right hemisphere normal. Arachnoidal Villi 
moderately developed. Pi A Mater shows injected 
veins, notably in the sulci of the right hemisphere. 
Pia mater everywhere thin and clear. Vessels at base 
of normal appearance. 

Brain weight 1180 grams. Spread on a board, the 
right hemisphere tends to flatten so that it measures 
1.5 cm, more from side to side than its fellow. Be- 
sides more marked venous injection, the right hemi- 
sphere shows also flatter and slightly more plastic 
convolutions. The posterior poles of the hemispheres 
are a little firmer than the parts anterior. The orbital 
and hippocampal gyri on the right side are a little 
firmer than the surrounding parts. On section the 
gray and white matter shows no lesions, excepting the 
slight plasticity of the tissues at large on the right side 
and a well marked induration, with retraction under 
the knife, of the occipital and hippocampal white 
matter. The basal ganglia of the left side are 
normal. On the right side a sagittal section demon- 
strates a rounded area of induration, with ill-defined 
borders, measuring perhaps 1.5 cm. from above down- 
wards by 2 X 2 cm., situated largely in the lenticular 
nucleus and involving the greater portion of the globus 
pallidus, a small segment of the putamen below and 
behind and the regionary part of the anterior com- 
missure with surrounding tissues. The most striking 
feature of this lesion is the occurrence in the middle 
of a cluster of vacuoles or cystic clefts, with smooth 
pale interiors, ranging from pin-head to 0.25 cm. or even 
0.5 cm. in greatest diameters. There are six to eight 
clefts to a surface of section. The color of the lesion 
differs little from that of the globus pallidus itself, but 
the tissue is a trifle translucent. It is impossible to 
demarcate the lesion with the eye. Induration is de- 
monstrable several mm. beyond the visible part of the 
lesion. The consistence of the lesion slightly surpasses 
the usual consistence of the olivary bodies. 

Cerrebellum, Pons and Bulb weight 165 grams. 
Cerebellar tissue a trifle more plastic than usual. 
The right olive is not so prominent as usual. 

Note. Thyroid: Weight 125 grams. Both lobes 
and isthmus enlarged. One lobe more than the other; 
lobe on one side measuring 6x4 cm. 



208 PUZZLES AND ERRORS 

Anatomical Diagnoses 

Enlargement of thyroid gland. 

Exophthalmos with dilated pupils. 

Fatty degeneration of thoracic muscles. 

Slight aortic sclerosis. 

Dilatation of right heart. 

Hypertrophy of left ventricle. 

Slight tricuspid endocarditis. 

Bicuspid aortic valve. 

Hypostatic pneumonia. 

Acute and chronic splenitis. 

Fatty liver (central necroses?). 

Acute nephritis. 

Chronic gastritis. 

Small breasts. 

Axillary hair absent. 

Petechial eruption of chest. 

Varicose veins. 

Chronic external adhesive pachymeningitis of left side. 

Moderate swelling of right hemisphere with venous 
injection. 

Slight occipital gliosis of both sides. 

Slight gliosis of orbital and hippocampal gyri of 
right side. 

Sclerosis with atrophy of occipital and hippocampal 
white matter of right side. 

Gllotic lesion (1.5 X 2 X 2 cm. of right lenticular 
nucleus involving anterior commissure). 

1. Was the exophthalmic goitre in Carrie Pearson due to 

syphilis? Unfortunately we have no clear proof that 
Carrie Pearson was syphilitic. She was stated to have 
been syphilitic by the physician who treated her before 
her commitment to Danvers Hospital, There Is, how- 
ever, no proof of syphilis, inasmuch as the patient died 
in the pre-Wassermann period. 

2. Is the thalamic lesion probably syphilitic? No lympho- 

cytosis or plasmocytosis characterizes the lesion, which 
is the only lesion of the sort In the Danvers collection. 
It would not do to call a lesion syphilitic just because 
it Is sui generis. In any event, the clinical analysis of 
the case faced the claim of syphilis as an actual factor 
in the patient's life and as a possible factor in the goitre. 



PUZZLES AND ERRORS 209 



It is well known that the ARGYLL-ROBERTSON 
PUPIL is characteristic of the so-called *' PARA- 
SYPHILITIC DISEASES" (''general paresis" 
and " tabes ") ; does this sign occur in other neuro- 
syphilitic conditions? 



Case 54. Julius Kantor was a shoemaker of 35 years, 
who came to the hospital for treatment because his family 
physician had found a positive W. R. in Kantor's blood 
serum. He had had a cough for a number of years, and 
during the last year a little blood had been found in the 
sputum; whereupon Kantor had been placed under active 
anti-tuberculosis treatment. The enterprising family physi- 
cian had found the positive W. R. in the first days of his 
treatment for tuberculosis. There was, in fact, a history 
of a chancre nine years before, which had not been followed 
by any secondary or tertiary symptoms, and which had 
been but scantily treated. 

There were no mental symptoms. 

Kantor was physically fairly well developed and nourished. 
There were a few piping r^les in the left upper chest, both 
in front and back, and also a slight dulness with increased 
vocal and tactile fremitus. No tubercle bacilli, however, 
could be found on repeated sputum examination. 

Neurologically, the pupils were myotic and both showed 
the Argyll-Robertson reaction. There were no abnormal 
reflexes whatever, and there was neither ataxia nor speech 
defect. Not only the blood but also the spinal fluid W. R. 
proved to be positive; there was a marked increase in the 
albumin and globulin; there was a gold sol reaction of the 
syphilitic type, and there were but three cells per cmm. 

I. In view of the headache in case Kantor, what other 
causes of headache are to be considered? It is cer- 
tain that Irritations of the dura mater can produce 
headache, and the physiological observation of the 
sensitiveness of the membranes and the non-sensitive- 



2IO PUZZLES AND ERRORS 

ness of the brain substance is an ancient and classical 
observation. Internal hemorrhagic pachymeningitis 
produces severe headache. The relations of this dis- 
ease to trauma, to arteriosclerosis, and possibly to 
syphilis (alcohol perhaps should also be considered) in 
certain instances have not been entirely cleared up. 
Syphilitic headaches are, according to Lewandowski, 
dependent also upon a dural affection or upon a perios- 
teal affection. The headaches of brain tumor are also 
commonly related to dural conditions, either directly 
due to the pressure of the tumor itself, or indirectly to 
the heightened intracranial pressure consequent upon 
the tumor. It is clear that the tension under which the 
dura mater lies is not always localized in the region 
of a brain tumor or a syphilitic lesion. Head has 
claimed that brain tumor produces headaches of two 
kinds, according to whether the disease affects the dura 
mater or is dependent upon an increase of pressure in 
the brain. It does not appear that the pia mater has 
any relation to headaches, but meningitis, in which 
the inflammation is confined to the pia mater, is never- 
theless associated with headache; the headache is here 
supposed to be due to the increase in brain pressure, 
and thus actually to an effect wrought upon the dura 
mater. Vasomotor disorders and various types of 
cephalic hyperemia are thought to produce a kind of 
headache, but Lewandowski calls this kind of head- 
ache somewhat in question. Reflex headaches are 
stated to be produced indirectly by a process of radi- 
ation from interior lesions in the brain. There are 
certain headaches called nodal headaches {Schwielen- 
Kopfschmerz). Hypermetropia, caries of the teeth, 
adenoids, and diseases of the nose and axillary cav- 
ities, to say nothing of thoracic and abdominal diseases, 
are also counted among conditions that may produce 
headaches. In this connection. Head has claimed 
differential zones of headache corresponding to certain 
diseases. 

The brain itself may produce headache through in- 
toxications, through conditions produced by a variety 
of diseases; may follow neuroses. Alcohol may pro- 
duce headaches in some persons even when it is taken 
in very small doses. Certain uremic cases yield head- 
aches, as do also gouty and chlorotic conditions. 
According to Lewandowski, the headaches of arterio- 
sclerotics are due possibly to vasomotor disturbances 



PUZZLES AND ERRORS 211 

in the membranes, or one may think of nutritive cere- 
bral disorders. A peculiar form of headache is that of 
fatigue after mental work,fallied to which is the neuras- 
thenic headache; constitutional headaches have been 
assumed to occur, to say nothing of hysterical head- 
aches. There remains also the important question of 
migraine, for which a vasomotor explanation has been 
proposed. 

2. Was Kantor suffering from tuberculosis of the lungs? 

The hypothesis of lung syphilis ought certainly to be 
very seriously considered. Upon repeated sputum 
examination, no tubercle bacilli have yet been found. 

3. Is Kantor a case of general paresis? In the absence of 

mental symptoms, and in consideration of the mild- 
ness of the reactions, it is certainly not easy to make 
the diagnosis of general paresis. However, the diag- 
nosis of tabes dorsalis is not justified either. Accord- 
ingly, we may answer our question: whether the 
Argyll- Robertson pupil occurs in other neurosyphilitic 
diseases, by pointing out that in the case of Julius 
Kantor, as in the case of Henri Lepere (105) and 
Frederick Stone (106), the Argyll-Robertson pupil has 
been found in syphilitic conditions that are neither 
typically paretic nor typically tabetic. 






212 PUZZLES AND ERRORS 



Does the Argyll-Robertson pupil necessarily indi- 
cate neurosyphilis? 



Case 55. Daniel Falvey, 44 years of age, was an alms- 
house transfer to the Danvers State Hospital in the year 1904, 
when the principle of state care was adopted in Massachu- 
setts. As in most of the almshouse transfers of that day, 
little could be discovered as to antecedents. He had been a 
mill-worker from the time of his immigration in 1890, at 30 
years of age. He had been somewhat alcoholic. There was 
a shock some 17 months before his death, which occurred 
about seven weeks from the date of transfer. 

Not only was he unable to walk unsupported, but when 
supported there was a slight dragging of the left leg and the 
gait was noted to be somewhat propulsive. The tongue and 
hands were tremulous, and the left grasp was somewhat 
weaker than the right. Both knee-jerks were increased 
although neither more than the other. There was no sensory 
disorder. 

Although but 44 years of age, Falvey presented the appear- 
ance of a much older man. His heart was somewhat enlarged 
and there was a degree of peripheral arteriosclerosis. On the 
whole, no special attention was attracted to this case clin- 
ically and he was regarded as an example of arteriosclerotic 
dementia, like many another among the transfers. However, 
we owe to Dr. H. M. Swift the important observation of the 
Argyll-Robertson pupils. The case was studied long before 
the Wassermann method was available, and is here reported 
merely to call attention to the fact that the stiff pupils may 
have other neural origin than neurosyphilis. 

The autopsy material in the case was worked up by one of 
the authors.* The autopsy had been performed by Dr. A. M. 
Barrett, who found on section through the brain stem at the 

* E. E. Southard. A case of glioma of the pineal region, 
Am. Jour, of Ins., Vol. LXI, 1905. 



PUZZLES AND ERRORS 213 

anterior border of the pons a mass springing from and contin- 
uous with the pineal gland, lying in the third ventricle and the 
aqueduct of Sylvius. Upon further study, this mass was 
found to begin posteriorly in the pineal body itself, from 
which the mass could hardly be told in the gross except by an 
injected border. 

This mass proved upon microscopic examination to be a 
psammoma, which histologically resembled a glioma rather 
than a sarcoma. Throughout the mass there was a variable 
content of fibrillary intercellular substance having the histo- 
logical reactions of neuroglia fibrillae. The histological 
details (mitosis, large giant cells with multiple nuclei, etc.) do 
not here concern us. We deal with a neoplasm springing from 
the pineal gland growing on the posterior half of the third 
ventricle, the anterior orifice of the aqueduct of Sylvius, and 
the space between the velum interpositum as far back as the 
posterior corpora quadrigemina. There is no evidence in the 
body of old syphilis; although it is possible that the stiff 
pupils were neurosyphilitic, it seems probable that they were 
related to the pineal tumor. At all events, there are in the 
literature evidences that the pineal-quadrigeminal group of 
tumors and other lesions may bring about pupillary distur- 
bances. On this account, we here Include the case. The 
tumor hardly led to an error In diagnosis since neither neu- 
rosyphilis nor brain tumor was at all expected clinically. 

1. Can alcoholism produce identical results? See Case 

Murphy, (60), one of alcoholic pseudoparesis. 

2. What is the nature of stiff pupils? A pupil is called 

stiff In the sense of the Argyll-Robertson pupil if it 
fails to react to illumination either of itself or of the 
other eye and at the same time if it reacts properly in 
convergence and accommodation. Of course the stiff- 
ness of a blind eye must not be regarded as an Argyll- 
Robertson pupil. In a case of right-sided Argyll- 
Robertson pupil, therefore, the left pupil reacts properly 
both to direct illumination of itself and to illumination 
of the right eye, but the right eye fails to react to illumi- 
nation of either eye. Such an Argyll-Robertson right 
pupil will remain of the same width both in darkness 
and in light. Clinicians agree that the Argyll-Robert- 



214 PUZZLES AND ERRORS 

son is diagnosticated rather too frequently than too 
seldom, and this by reason of the fact that a sluggish- 
ness of light reaction is interpreted as stiffness. The 
sign, as is well known, has come to be regarded as almost 
pathognomonic of tabetic or paretic neurosyphilis. 
Nonne, however, has found among 510 cases of alco- 
holism, nine instances of Argyll- Robertson pupil and 
19 cases of sluggish light reactions. The pathological 
anatomy of this sign is still doubtful although a number 
of schematic accounts are available ; among hypotheses, 
one may think of an elective effect of the tabetic or 
paretic degeneration upon reflex collaterals. The ex- 
planation would then resemble that for absent knee- 
jerks and kindred reflex disorders. We should then 
hypothesize a loss of the finer processes of the terminal 
aborizations about the cells of the nucleus of sphincter 
nucleus iridis. However, the situation of the sphincter 
iridis has not yet been absolutely determined. 

When a pupil is said to be entirely stiff it means that 
it reacts neither to light nor accommodation. This 
condition not infrequently follows the partial stiffness 
or Argyll- Robertson reaction. 
3. Is the Argyll- Robertson pupil more tabetic than paretic? 
This has been claimed at times, but in point of fact, the 
Argyll- Robertson pupil is very frequent in paresis, and 
so also are posterior column changes. According to 
statistics of Bumke, 36% of tabetics fail to show the 
Argyll-Robertson pupil, and 38% of paretics. When, 
however, finer methods, such as those standardized by 
Weiler, with photographic records, are employed, the 
number of cases without at least a tendency to the 
Argyll- Robertson pupil becomes much smaller. 

In connection with the important question as to the 
classical Argyll- Robertson pupil and pupillary slug- 
gishness to light, it may be inquired what are the ocular 
signs in neurosyphilis? Joffroy has tabulated the signs 
in 300 general paretics as follows: 

Sign. No. of cases. Per cent. 

Alterations of light reflex 235 78 

Inequality 205 68 

Abolition of light reflex 156 52 

(bilateral or unilateral) 

Abolition of light reflex 133 44 

(bilateral) 

Irregularity of pupil 117 39 



PUZZLES AND ERRORS 215 

Sign. No. of cases. Per cent. 

Irregularity of both pupils 109 36 

Diminution of light reflex 108 36 

ditto (bilateral) 79 26 

Alteration in accommodation reflex. 79 26 

Diminution of accommodation reflex 52 17 

Mydriasis 41 13 

Myosis 40 13 

Diminution of light reflex 35 11 

(unilateral) 

Abolition of accommodation reflex. . . 35 11 

Diminution of accommodation reflex 29 9 

(bilateral) 

Abolition of accommodation reflex . . 26 8 

(bilateral) 

Diminution of accommodation reflex 23 7 

(unilateral) 

Fundus changes 21 7 

Vascular changes 16 5 

Abolition of accommodation reflex. . . 12 4 

(unilateral) 

Paresis of the third nerves 10 3 

Ptosis 9 3 

Irregularity of one pupil 8 3 

Nystagmus 7 2 

Visual acuity lost 7 2 

Atrophy of disc 6 2 

Total blindness 5 2 

Paralysis of the fourth nerves i I 



2l6 PUZZLES AND ERRORS 



Can neurosyphilis exist in the absence of positive 
findings in the spinal fluid? 



Case 56. There was no great difficulty in setting up a 
diagnosis of general paresis in the case of James Burns, a 
mechanic of 31 years of age, who came voluntarily to the 
Psychopathic Hospital for treatment. The point in Burns' 
case was that the spinal fluid proved entirely negative in all 
respects despite the fact that the serum W. R. was positive, 
and despite the following facts of history and mental exami- 
nation. 

The patient claimed syphilitic infection seven years before, 
namely, at 24 years of age, and also claimed that he had 
infected his wife, who was in fact at the time undergoing anti- 
syphilitic treatment. He complained of insomnia, worry, 
depression, hypersensitivity to noises (such as those made 
by his own children) , thoughts of suicide, and amnesia. The 
amnesia, however, might be regarded as subjective since our 
tests failed to show amnesia. Nor was there any diminu- 
tion in arithmetical ability. Despite the patient's claim that 
he had been " way off in his way of thinking," there appeared 
to be no delusions. Beyond a certain flightiness in conversa- 
tion, we could hardly get any evidence of psychosis unless of 
the neurasthenic order. 

Physically, however, the left pupil failed to react to light 
though it was found to react to distance, and the right pupil 
exhibited a diminution of its reaction to light. There was 
no ataxia of gait, yet there was a complete Romberg reaction. 
There was a moderate tremor of the hands and of the tongue. 
Otherwise there were no reflex disorders upon systematic 
examination, nor was there any demonstrable disorder in the 
rest of the physical examination. 

I. What is the diagnosis in the case of James Burns? ^On 
the whole we agree with Nonne, that negative spinal 
fluid findings (of course, in the absence of treatment) 
preclude the diagnosis of general paresis. The symp- 



PUZZLES AND ERRORS 217 

toms might possibly be explained, however, by means 
of a localized syphilitic involvement of the cerebrum, 
no cells or products of inflammation having penetrated 
to the spinal fluid. According to Head and Fearnsides, 
this condition may be found especially in the anterior 
or middle fossa. Accordingly, going upon these views 
of Nonne and of Head and Fearnsides, we should be 
entitled to make, perhaps, a diagnosis of cerebral 
syphilis. 
2. What is the significance of the Argyll- Robertson pupil in 
James Burns? Nonne states that if one follows cases 
with Argyll- Robertson pupil over a sufficient period 
of years, they one and all eventuate in active symptoms 
of cerebrospinal syphilis (not necessarily of the cortical 
type) , and this despite the fact that the pupillary change 
may have been present a number of years before any 
other symptom had developed. 



2l8 PUZZLES AND ERRORS 



Neurosyphilis ('' DISSEMINATED ENCEPHA- 
LITIS") within seven months of initial infection. 
Autopsy. 



Case 57. We borrow the main features of a remarkable 
case examined at the Danvers State Hospital clinically by 
Dr. H. W. Mitchell and reported elaborately by Dr. A. M. 
Barrett. This case, whom we shall call John Summers, 
acquired syphilis at about the end of the third week in May, 
1902, and consulted a physician on June 12, at which time 
a characteristic initial lesion of syphilis was plain. Summers 
was excessively alcoholic at times and was not seen by a 
physician again until July 2, just after an alcoholic debauch. 
At this time there was ulceration of the primary lesion, and a 
papillary eruption had developed over the arms, chest, 
abdomen, and legs. Mercurial treatment and mixed treat- 
ment were given. Arthritis occurred but disappeared with 
increased dosage. 

About six months after infection, the patient developed 
severe headaches, hardly controllable by treatment. Amnesia 
and a certain stupidity, with neglect of personal habits, and 
even of eating, developed, whereupon Summers was admitted 
to the Danvers Hospital, December 11, 1902. He weighed 
124 pounds, was extremely feeble, with dull and expression- 
less face, coarse purposeless movements of arms; left pupil 
larger than right; right external strabismus and ocular ptosis; 
increased knee-jerks, crossed adductor reflex, coarse tremors 
of arms and hands ; and extreme clguding of consciousness. 
It was doubtful whether the pupils were stiff to light or not. 

The patient died on the ninth day, December 18, in a 
state of coma. After admission, his stupor had become more 
marked; there had been incontinence of urine and faeces, 
and the patient could be aroused only by loud tones. 
Difficulty in swallowing had developed; the right-sided 
ptosis had become more marked, and muscular twitchings 
had developed on the right side. When the left leg was 



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2. Superficial (subpial) cellular 
reaction of neuroglia tissue (expand- 
ed cell bodies). 



3. Cellular gliosis of deeper 
layers of cortex. Apparent in- 
crease in capillary supply, possibly 
relative to loss of neural elements. 



Case 57. Neurosyphilis ("disseminated syphilitic encephalitis" of A. M. Barrett), fatal 
seven months from initial infection. (Photographs by A. M. Barrett.) 



PUZZLES AND ERRORS 219 

pinched, there was twitching of the left leg and arm. There 
was slight spasticity of the right arm and leg. An examination 
upon the day of death definitely showed a lack of reaction of 
the pupils to light. 

Dr. Barrett was able to find in the literature a case of 
Bechterew which histologically resembled his own case, but 
though in the instance reported by Bechterew the first symp- 
toms developed within the year following infection, death did 
not occur until two years later. 

In view of a total duration of symptoms clearly not over 
seven months, it is interesting to inquire how far micro- 
scopic brain changes could have proceeded. Neither cal- 
varium nor dura mater showed changes. There was a slight 
haziness of the pia mater over the convexity, but the pia 
mater over the base (especially below the cisterna and from 
thence spreading out over the pons and into the fissure of 
Sylvius) was not only hazy but definitely thickened and 
hypersemic. The thickening was most marked about the 
root of the right third nerve (corresponding with the eye 
findings in life) . There was also a macroscopic thickening of 
the left Sylvian artery. Section of the brain showed nothing 
abnormal except a small area among the pyramidal fibres 
of the right side of the pons, where there was a single hemor- 
rhagic area about 7 mm. in diameter around which there 
were small punctiform hemorrhages. (Compare twitchings 
of left leg and arm upon stimulation of left leg, and note 
also the muscular twitchings and slight spasticity of right 
leg and arm noted just before death.) This case was exam- 
ined and reported upon in 1905. We learn from Dr. Barrett 
that a re-study of the case with modern methods has failed 
to demonstrate a spirochetosis. 

The meninges show infiltration and destructive and pro- 
liferative changes of the blood vessels. Condensed extracts 
from Dr. Barrett's full report follow: 

There were local variations in the severity of the 
meningitis. The sulci showed the most marked in- 
filtration. The slighter degrees of exudation were 
made up largely of lymphocytes with a few plasma 
cells, occasionally large mononuclear cells, and rarely 



220 PUZZLES AND ERRORS 

a polymorphonuclear leukocyte. Where the exudation 
was more extensive, the large mononuclear cells became 
more common and the polymorphonuclear leukocytes 
increased in number. The large mononuclear cells were 
often phagocytic, containing from one to six leukocytes. 
The exudate was always most abundant about the 
blood vessels. The plasma cells were always most 
numerous in the adventitia of the veins, here greatly 
outnumbering the leukocytes. The polymorphonuclear 
leukocytes were relatively infrequent except where there 
were necrotic areas, which areas were usually con- 
tinuous with an infiltration of a vessel wall. 

As to vascular changes, the media was not often 
involved, nor was the adventitia so often affected as the 
intima. Such lesions as appeared in the intima and 
adventitia were infiltrative rather than proliferative. 
The elastica of the blood vessels proved to show but 
slight changes. 

A characteristic change was the endarteritis, — of a 
focal nature with a few large mononuclear and lympho- 
cytic cells pushing the intima inward at the edge of a 
lesion. In the more marked portion of the focal process, 
the thickness of the intima was greatly increased by 
proliferation. Great numbers of large mononuclear 
cells could be seen between the intima and the elastica. 
About these cells and interlacing among the other 
elements of the proliferating tissue was an excess of 
connective tissue fibres. 

The meningeal veins were more often diseased than 
the arteries; there was adventitial infiltration with 
lymphoid and plasma cells; sometimes the vein walls 
had become necrotic and infiltrated with polymorpho- 
nuclear leukocytes. 

It will be remembered that the left Sylvian artery 
was grossly thickened, and microscopic section of this 
vessel showed a partial thrombosis. 

The uram showed diffuse and focal changes. The 
diffuse process was one of nerve cell degeneration and 
proliferative changes in the neuroglia and blood vessels, 
and no section of the many examined proved to be free 
from such changes, although in the majority of instances, 
these diffuse changes were slight. The cortical layers 
showed more of these diffuse changes than did the 
white substance. Barrett considered that the glial cell 
changes were more delicate indicators of the cortical 
changes than the nerve cell changes. He found rod 





V 



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rr- 



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5. Focal vascular lesions. 
Case 57. Seven months from infection. "Disseminated syphilitic encephalitis," Barrett. 
(Photographs by Barrett.) 




Paretic neurosyphilis ("general paresis") — cerebral atrophy, without 
meningitis. Therapeutics cannot hope to restore lost tissue. 

Duration. 3 years from beginning of well marked symptoms; 6 years 
from beginning of obvious symptoms; 12 years from a so-called " nervous 
prostration." 



PUZZLES AND ERRORS 221 

cells, satellitosis, superficial gliosis, and a large gamut 
of changes in the neuroglia. There were two rather 
characteristic nerve-cell changes: a shrinkage change 
going on to almost complete destruction, and a type of 
cell swelling, also apparently proceeding to complete 
destruction. 

Among focal changes, there were four main types: 
Areas of encephalitis, having the general appearance 
of granulation tissue, areas of simple necrosis or soften- 
ing, apparently directly related to vascular changes 
near by, hemorrhages, and certain foci regarded as 
gummatous. 

Save for pial infiltration and a few vascular changes, 
there was very little change in the medulla and spinal 
cord. There was a hypertrophic gliosis of the margin 
of the medulla and cord throughout, and a focal lesion 
of well-marked gliosis at one point in the bulb. There 
were no fibre degenerations in the medulla or cord, nor 
were there any coarse fibre degenerations in the cortex 
itself except in one locus, the left gyrus rectus. This 
case is of peculiar value in showing to what extent 
lesions may proceed in a period of six to eight months 
after primary infection. 

Of course the case is in one sense entirely atypical. The 
lesions were not confined to the nervous system. Aside from 
the maculo-papular eruption and ecchymosis of the skin, 
there was a diffuse hemorrhage of the inner half of the con- 
junctiva of the left eyeball, a small hemorrhagic focus in the 
mitral valve, a caseous nodule, one cm. in diameter, in the 
apex of the left lung whose tuberculous or syphilitic nature is 
left in doubt; a broad milk-colored patch of thickening of 
the capsule of the liver. It is to be noted that there were no 
gross lesions of the aorta. 



222 PUZZLES AND ERRORS 



On the classical assumption that PARETIC NEU- 
ROSYPHILIS ("general paresis") is a fatal 
disease, is there a disease PSEUD OPARETIC 
NEUROSYPHILIS (" pseudoparesis ") which may 
recover or pursue a long course like that of a case 
of diffuse neurosyphilis (" cerebrospinal syphilis ")? 



Case 58. Peter Burkhardt had been an efficient highway 
inspector, but in his forty-fifth year he had begun to be unable 
to do his work satisfactorily. His wife had become somewhat 
afraid of him. He had had somnolent spells in his chair and 
had squandered money. The mental symptoms had lasted 
for some six months, but had become more marked during 
the month preceding admission. Burkhardt would at times 
fail to recognize his friends. 

The general physical condition of Burkhardt was very good. 
The pupils were irregular and reacted sluggishly both to light 
and to accommodation. The knee-jerks and ankle-jerks were 
absent. There were no other neurological disorders upon 
systematic examination. There was a speech defect. Men- 
tally, little could be determined except a certain sluggishness. 

History and physical examination at once suggested gen- 
eral paresis. The serum W. R. was doubtful, but the spinal 
fluid reaction was positive, as was the gold sol reaction (which 
was "paretic"); the globulin and albumin were greatly 
increased; there were 48 cells per cmm. Antisyphllltic 
treatment, consisting of salvarsan twice a week and potassium 
iodid by mouth, was followed by a rapid mental Improve- 
ment. After two months, Burkhardt was discharged appar- 
ently normal, with all the blood and spinal fluid tests neg- 
ative. He has been taken back Into the highway service. 

I. What is the proper definition of pseudoparesis? Fournler 
termed pseudoparesis certain cases that looked like 
paresis but were not syphilitic In origin. Of these 
cases the most characteristic group Is that of alcoholic 
pseudoparesis. It is clear that there will be no diffi- 



PUZZLES AND ERRORS 223 

culty in the definition of a disease pseudoparesis whose 
entity is presented in the adjective that precedes the 
term {e.g., alcohoHc pseudoparesis). According to this 
usage, a case of pseudoparesis would be one in which the 
symptoms and possibly some of the signs somewhat 
resemble the symptoms of paresis itself but for which 
another etiology could be fairly established. 

2. Are there any cases of syphilitic pseudoparesis? We are 

of the opinion that the term should be dropped. It is 
true that there are cases which clinically look like gen- 
eral paresis and exhibit the appropriate laboratory signs 
of general paresis but seem to differ from paresis in their 
course even when they receive no treatment whatever. 
In the present phase of doubt as to the classification of 
paretic and non-paretic forms of neurosyphilis, it seems 
to us of doubtful utility to characterize a case as pseudo 
simply because it differs in its course, particularly as 
the literature has always duly recognized that a number 
of cases of general paresis have had long courses and 
sometimes very long remissions. 

There is also another group of cases that have been 
termed cases of pseudoparesis, namely: certain cases of 
neurosyphilis which clinically look like general paresis 
and seem to be following its classical course but are 
interrupted by treatment. Here again it seems to us 
doubtful whether the designation pseudo should be 
attached to this group of cases, particularly while the 
whole therapeutic question in the paretic group of neuro- 
syphilis cases remains sub judice. Accordingly we are 
tempted to include in the group of paretic neurosyphilis 
cases that either get well of themselves, or get well under 
treatment, or pursue a very long course, or are subject 
to very long remissions. But we make this decision in 
terminology without prejudice to the therapeutic ques- 
tion and it is open to any critic to throw these cases into 
an atypical non-paretic group of neurosyphilis cases. 

3. How shall we explain the absence of ataxia of case Burk- 

hardt when knee-jerks are absent and when, therefore, 
we are entitled to conclude a certain degree of spinal 
disease? As stated in connection with case Sullivan 
(16), the absence of knee-jerks is not a warrant for 
terming a case — paresis of the tabetic form. The fact 
is that the lesion in paresis tends to be intraspinal, 
just as the higher brain lesions tend to occur within the 
brain substance. The meninges are relatively spared 
both within the cranium and within the spinal canal. 



224 PUZZLES AND ERRORS 

The characteristic degeneration of posterior nerve 
roots which we find in tabes dorsalis is not necessarily- 
found in general paresis even when there are somewhat 
extensive spinal lesions. Accordingly the absence of 
sensory returns by way of the posterior nerve roots 
which characterizes tabes dorsalis is not necessarily a 
phenomenon of general paresis. The mechanism by 
which the knee-jerks are lost depends upon histo- 
logical detail. They may be lost when under tabetic 
conditions the posterior roots are severely diseased 
and when under paretic conditions only intraspinal 
collaterals or a small portion of fibres are affected. 
The whole question hinges upon where and to what 
degree the various reflex arcs are cut in the disease. 
The tabetic phenomena are, as so commonly stated, 
intradural ; that is, the sensory or gangliospinal neurones 
at certain levels are affected all the way in from the 
points at which they pierce the dura mater. The affec- 
tion of these and other neurones in general paresis is 
an intraspinal and parenchymatous affection. 



PUZZLES AND ERRORS 225 



Neurosyphilis; auditory hallucinations; ideas of 
persecution ; attacks of excitement. SYPHILITIC 
PARANOIA (Kraepelin)? 



Case 59. Bridget Curley was a case that was discharged 
from the Psychopathic Hospital, recovered, after 26 days in 
hospital. The symptoms so resembled those of alcoholic 
hallucinosis that the diagnosis was made despite the fact that 
the patient consistently denied the use of intoxicants. There 
was, in fact, no proof that she drank alcohol. The case was, 
however, not clearly one of alcoholic hallucinosis or of any 
other well-defined form of mental disease. A provisional 
diagnosis of manic-depressive psychosis, manic phase, had, in 
fact, also been made. 

The illness had begun with depression and inactivity, 
Bridget's friends accounted for these conditions on the ground 
that a lover had departed for Ireland. A few days after the 
depression began, Bridget became dizzy and refused to give a 
boarder his breakfast, stating that she had lost her memory 
and had begun to hear bells ringing and people talking. She 
then became greatly excited and was brought to hospital, 
where the prolonged baths quieted her. 

It seems that Bridget had had stomach trouble and head- 
aches at the top of her head or sometimes in her temples. 
Physical examination showed the left pupil to be larger than 
the right, a slight tremor of the lips, a slight systolic murmur 
at the apex, slightly irregular pulse, and moderate edema of 
ankles. The blood serum was negative to the W. R., but 
lumbar puncture was executed and the fluid showed a posi- 
tive W. R. 

The patient was tested by the Binet and other methods, 
and although 35 years of age, seemed to be by the mental 
tests hardly over 11 years old. She was inclined to be fe- 
verish, somewhat restive, and pugnacious; rather slow of 
speech, sometimes refusing to answer and grimacing. Her 
pugnacity was, however, easily controllable, and the excite- 



226 PUZZLES AND ERRORS 

ment was largely at night. This excitement subsided rapidly 
in the course of a few days. 

1. What is the diagnosis in this case? The following diag- 

noses and suggestions for diagnosis were made at the 
staff meetings: 

Unclassified mania. 

Manic-depressive psychosis, manic phase. 

Toxic delirium. 

Dementia praecox. 

Bacterial infection of the brain. 

Unclassified delirium. 

Acute delirium. 

Infectious psychosis. 

Acute confusional psychosis. 

Psychopathic personality by use of alcohol. 

Mental deficiency with atypical mental state. 

Syphilitic paranoia. 

2. Is this a case of syphilitic paranoia? The so-called syph- 

ilitic paranoia of Kraepelin is a rare and uncertain type 
of syphilitic mental disease. Delusions and hallu- 
cinations are prominent. As a rule, the onset is stated 
to be slow and insidious, or at any rate there are a 
variety of indefinite prodromata. Jealousy is a prom- 
inent feature, sometimes attended with marked sexual 
excitement. Auditory hallucinations and ideas of 
persecution are particularly in evidence. The most 
striking feature in Kraepelin's group was a sudden oc- 
currence and equally sudden disappearance of violent 
excitement, with or without external cause. Thus, 
an excitement would be produced by a few words 
spoken, and immediately after, the phase of excitement 
would pass and the patient would become entirely 
friendly and accessible once more, as if nothing had 
happened. About half of Kraepelin's cases showed a 
positive serum W. R. He does not report lumbar 
puncture findings, and grounds the existence of disease 
upon certain autopsied cases. The speech and writing 
disorder of paresis as well as the characteristic disori- 
entation for time and muscular weakness of general 
paresis were absent in the group. It appears that 
most cases of the group have hitherto been placed in 
dementia praecox. 



PUZZLES AND ERRORS 227 



The clinical symptoms of CHRONIC ALCOHOL- 
ISM are sometimes largely identical with those of 
PARETIC NEUROSYPHILIS (''general paresis") : 
differentiation by means of the laboratory findings. 



To demonstrate this proposition, the cases of Francis 
Murphy (60) and David Collins (61) are in point, being 
sharp foils to one another. 

Case 60. A laboring man about 44 years of age was brought 
to the Psychopathic Hospital one summer day, in a stupor. 
This patient, Francis Murphy, had been at his regular work 
as axeman in the Park Service, when he suddenly fell in a 
heavy convulsion. He was carried to a general hospital, 
still in convulsions, and ether was administered to quiet 
the movements. The convulsions shortly ceased, but the 
patient's consciousness failed to clear; hence his transfer to 
the Psychopathic Hospital. 

Here he remained much disturbed and was placed in a room 
with a mattress on the floor. On this mattress he would 
crouch on all fours for a considerable time, looking fixedly 
downward as if at an object on the floor, unresponsive to 
questions but compliant with efforts to place him on his back. 
He gave the impression of daze and either disorientation or 
confusion. 

Within twenty-four hours the patient became more tran- 
quil and consciousness became clearer, but the patient was at 
a loss to bring to memory either recent or remote events. 
However, he replied to questions, giving some different story 
each time he was approached. Curiously enough, the patient 
seemed very contented and good natured and would even 
laugh foolishly at times, saying that he felt fine and all 
ready to go out to work. 

The general impression conveyed by Francis Murphy at 
once suggested the possibility of neurosyphilis. Convul- 
sions, perhaps initial in middle age, with a post-convulsive 



228 PUZZLES AND ERRORS 

stupor, followed by a partial clearing up, with persistent 
amnesia and a suggestion of fabrications with euphoria, bore 
out the suggestion. 

The physical examination strengthened the impression 
of neurosyphilis. Well developed and nourished, florid, with 
a manual tremor and sweating of the palms, the patient 
was in general without physical symptoms. Neurologically, 
however, whereas the left pupil was larger than the right and 
reacted properly to light, the right pupil was a bit contracted, 
somewhat Irregular, and either reacted not at all to light or 
very slightly so (reacting perfectly to accommodation). The 
knee-jerks could be obtained only with reinforcement, and 
several other reflexes could not be elicited (triceps, radial, 
ulnar, periosteal, Achilles, umbilical). Moreover, the heel-to- 
knee test was poorly performed; some of the common tests 
phrases were very poorly repeated ; there was marked tremor 
in writing; and the paragraphia seemed to be not merely 
peripheral, for syllables were left out in words and ordinary 
words spelled incorrectly (psychographic disturbance). 

We do not care here to Insist that the right pupil was really 
an example of the Argyll-Robertson phenomenon since the 
slightest tinge of doubt is important if a positive diagnosis 
is practically equivalent to asserting syphilis. Practically, 
however, the right pupil was regarded as an Argyll-Robertson 
pupil under hospital conditions (flash-light reaction). Argyll- 
Robertson pupil, areflexia, speech disorder, writing disorder, 
memory disorder, conduct disorder, and euphoria, all with a 
history of convulsions, certainly warranted the tentative diag- 
nosis of neurosyphilis. 

As usual, resort was made to the W. R. in the serum and in 
the spinal fluid. One of the first results to come through 
from the laboratory was the absence of globulin, normal 
albumin, negative gold sol reaction, and a cell count of two 
cells per cmm. in the spinal fluid. Later the W. R.'s were 
returned negative for blood and spinal fluid. 

In the meantime, an Illuminating change had occurred in 
the patient, for two days later, — three days after the first 
convulsion in the park, — the patient had apparently quite 
recovered; his consciousness became nearly clear; he could 



PUZZLES AND ERRORS 229 

remember every event up to the time of the convulsion, and 
his memory came back in appropriate degree for both remote 
and recent events. 

The patient, it appeared, had for some time been drinking 
more and more heavily. In recent days, he had been taking 
five or six whiskeys and a half dozen beers daily on the 
average, and often much more. About ten years before, the 
patient narrated, there had been a convulsion at a ballgame, 
and this convulsion the patient himself called a " rum fit." 

Here, then, is a case of Alcoholic Pseudoparesis. With- 
out the W. serum test and without the spinal fluid examina- 
tion, it is probable that the diagnosis of general paresis might 
have clung to the patient for some time on account of the 
apparent Argyll- Robertson pupil, which had to be accepted 
as such on the fiash-light data. In point of fact, in this case 
the pupil later reacted more normally to light, and the speech 
and writing disorders measurably cleared up. 

1. Can alcohol produce the Argyll-Robertson pupil? The 

majority of neurologists would today answer. Yes. 

2. If in the case of Francis Murphy, the W. R. in the blood 

had happened to be positive on account of a non-neural 
syphilitic infection (spinal fluid negative), would the 
diagnosis general paresis be warranted? Probably the 
diagnosis general paresis would have been made. If 
the patient had been lost to observation, he might 
well have been regarded as an atypical paretic with 
prodromal convulsions. 

3. Would positive globulin and excess albumin in the spinal 

fluid alone or in association with a positive serum W. R. 
warrant the diagnosis general paresis or neurosyphilis? 
The chances are that most neurologists would advocate 
proceeding to treatment in any case of positive serum 
reaction, whether or not there was globulin or excess 
albumin; but the positive globulin and excess albumin 
would probably not warrant the diagnosis general 
paresis or neurosyphilis in the absence of excess cells 
and the characteristic gold sol reaction and W. R. in 
the fluid. 

4. Is the case of Francis Murphy one of alcoholic epilepsy 

(as suggested by Murphy's own phrase, " rum fits ")? 
It must be remembered that epileptics become alco- 
holic and that epileptic convulsions increase or become 



230 PUZZLES AND ERRORS 

more severe with alcoholism. On the other hand, the 
literature indicates that alcoholism can produce con- 
vulsions, as can many other factors. The literature 
also indicates that there is a condition of epilepsy in 
which the convulsive tendency sets in as a result of 
alcoholism in a patient not previously disposed to epi- 
lepsy; it appears also that sometimes, though very 
rarely, the epilepsy continues after withdrawal of alcohol, 
and even after giving up the habit. Francis Murphy 
appears to have had but two spells of convulsions, both 
of them following heavy bouts with alcohol. There is 
so far, then, no warrant for calling Francis Murphy's 
case one of alcoholic epilepsy. 
5. Does the use of alcohol by a subject destroy the value 
of the W.R.? It has been held by some that alcohol- 
ism interferes with the accuracy of the W. R. This 
has not been our experience and for the present we 
are of opinion that the results have the same value 
in alcoholics as in non-alcoholics. The next case 
(Collins, 61) is one in which a positive W. R. occurred 
in an alcoholic. When dealing with paretic neuro- 
syphilis it is especially true that the W. R. is disturbed 
very rarely, if at all, by toxins or drugs, except anti- 
syphilitic drugs. 



PUZZLES AND ERRORS 23I 



ALCOHOLISM may cloud the diagnosis of NEURO- 
SYPHILIS. Differentiation by laboratory tests. 



Case 61. David Collins was a steamfitter of about 43 
years of age, picked up at 6.45 a.m. in the midst of convulsions 
and talking incoherently, in a state apparently of fairly clear 
consciousness. On arrival at the hospital, the patient was 
able to tell how he had always been a hard drinker, and how 
during the past week of unemployment he had taken large 
quantities of poor whiskey, — perhaps an average of a pint a 
day. Collins also told how he had had delirium tremens sev- 
eral times, but he said the present spell was quite unlike de- 
lirium tremens. There was no disorientation or impairment of 
memory, and the patient did not in any wise suggest a mental 
case a few hours after admission. 

It appears, according to Collins, that he had obtained some 
work the night before, and had quit work about 6.30, where- 
upon he stepped into a barroom, took one drink of whiskey, 
left the barroom, walked down the street, and suddenly lost 
track of the world, coming to consciousness in a carriage with 
two policemen, but remaining, as he said, " dopy," inatten- 
tive, and confused. After a meal, however, the patient began 
to feel better and soon felt quite all right. 

The physical examination was quite negative except that 
neurologically there was lingual and manual tremor, a speech 
defect, apparent only with test phrases, unsteadiness of hand- 
writing, left knee-jerk greater than right, a left-sided Babinski 
reflex, and a difficulty in executing rapid successive move- 
ments (dysdiadochokinesis) . This degree of neurological dis- 
order in our experience warrants lumbar puncture as well as a 
serum test. The lumbar puncture shortly disclosed a positive 
globulin and excess albumin, and the returns from the W. R.'s 
were positive for both spinal fluid and blood serum. The 
data of the gold sol reaction were not available on account of 
technical difficulties. However, it appears that the diagnosis 
of neurosyphilis could hardly be avoided in this case. 



232 PUZZLES AND ERRORS 

David Collins differs from Francis Murphy, then, in show- 
ing a positive blood and spinal fluid reaction for syphilis as 
well as a positive globulin and excess albumin. As above 
remarked, it is probable that the positive globulin and excess 
albumin would not warrant more than a suspicion of neuro- 
syphilis taken by themselves. 

Unfortunately, we were unable to persuade the patient to 
submit to treatment, and from the patient's point of view 
possibly his decision, not to submit to treatment, was a good 
one since he has had no symptoms of any sort for a period of i8 
months since his episode. However, as abundantly else- 
where demonstrated, we feel that the patient is wrong, and 
that the physicians are right in urging treatment. 

1. Is not the convulsive episode an alcoholic phenomenon 

in David Collins entirely separate from the patient's 
general and neurosyphilis? Possibly; however, an 
outbreak of neurological symptoms with spontaneous 
recovery is not only consistent with the diagnosis of 
syphilis, but somewhat characteristic of neurosyphilis. 
We suspect that another attack will occur In David 
Collins,* We shall from time to time make use of the 
social service to suggest his going under treatment, and 
shall employ his record of contact with a public institu- 
tion to drive in our suggestion. Still it is clear that 
there are numerous cases In the community that are 
not accessible to social service initiated from a public 
institution. Accordingly, educational propaganda is 
necessary for salvage of the middle- and upper-class 
victims of syphilis. It Is a little unfortunate that the 
ethics of the private practitioner make such salvage of 
middle- and upper-class persons not very likely. Might 
it not be that an extension of state medicine to this 
field would incidentally Increase the amount of success- 
ful private practice? 

2. What may be the cause of such a convulsive episode as 

that of David Collins? It would appear that the con- 
vulsions of general paresis and of neurosyphilis in gen- 
eral often occur without gross structural lesions of the 
brain. It may be suggested that vascular Irritation or 

* Since this was written Collins has had further difficulties 
related to his neurosyphilis, improving under treatment. 



PUZZLES AND ERRORS 233 

parenchymal irritation by spirochetes, acting in appro- 
priate parts of the central nervous system, can produce 
such convulsions. 

3. What is the significance of the unilateral phenomenon in 

David Collins (left knee-jerk greater than right; left- 
sided Babinski)? The current explanation of hyper- 
refiexia is that somehow inhibitory impulses from upper 
portions of the nervous system have ceased to influence 
the local arcs that mechanize reactions like the knee-jerk 
and the normal plantar reflex. The phenomena are 
commonly found in cases with pyramidal tract disorder, 
and in the case of David Collins one may suspect, there- 
fore, that there was a central disorder affecting the right 
pyramidal tract above its decussation. One might 
suspect that the convulsions were initiated by a lesion 
(whether gross or microscopic in range) in the right 
side of the cerebrum ; but whether in the white matter 
or in the gray matter must be left doubtful. The 
clearing up of all symptoms suggests either that the 
lesion was microscopic in range or that the phenomena 
were transient and functional. 

4. Can the dysdiadochokinesis be used to indicate cerebellar 

lesion in David Collins? Possibly; but it does not 
appear that the difficulty in executing successive move- 
ments was unilateral. It seems impossible to bring 
into close topographical relation the basis for the 
Babinski and left-sided hyperreflexia, and the basis for 
the dysdiadochokinesis. Alcohol is sometimes asserted 
to exert an especial effect upon the cerebellum. 

5. Must we suppose structural lesions, either (a) of the 

nature of cell losses demonstrable microscopically, or 
(b) of the nature of secondary degenerations demon- 
strable by Weigert myelin sheath methods, in the case 
of David Collins? It appears that we do not need to 
assert the existence of such lesions. 

6. Could the hyperreflexia and the Babinski reaction be due 

to local spinal cord disease? Possibly ; but the existence 
of other neurological symptoms (lingual and manual 
tremor, speech defect to test phrases, ataxic hand- 
writing, and dysdiadochokinesis) makes it probable 
that there were lesions, or at any rate disordered func- 
tions, within the cranium; and there appears to be no 
basis for asserting local spinal cord disease. 



234 PUZZLES AND ERRORS 



Differential diagnosis between NEUROSYPHILIS 
and ACUTE ALCOHOLIC PSYCHOSIS. 



Case 62. Joseph Buck was a chef of 60 years who came in, 
seeking advice because his memory was getting poor; he 
was unable to remember names and what he was about to do. 
He was tremulous and had much pain in his limbs. He had 
been drinking heavily for weeks, — probably ten weeks; 
in fact, he described himself as having had " the shakes " 
and as having lately seen animals and people that were unreal. 
He had had the shakes before and the condition had lasted 
for two to three days after alcohol was discontinued. 

Physically, Buck was tall, well developed, although poorly 
nourished, with a skin suggesting alcoholism. There was 
a slight acne over the back and chest; there was a slight 
enlargement of the heart, with blood pressure, systolic, 180, 
diastolic, 120. There was a corneal opacity of the left eye, 
which the patient said was the result of syphilis following a 
chancre, which he had acquired at the age of 27. There 
was also a ptosis of the upper lid of the left eye. The right 
pupil was irregular and reacted to light sluggishly, and with 
a very small excursion. The patient was slightly deaf in 
both ears. The deep reflexes were all lively and equal. The 
tremor was most marked in finely coordinated movements. 
There was a slight swaying in the Romberg position but the 
sign could not be said to be present. The gait was unsteady. 
There was a marked tenderness over the nerve trunks. 

So far as mental examination went, it seemed that the 
patient's claim of amnesia was subjective. There was cer- 
tainly no more amnesia than a slight difficulty in recalling 
details. The diagnosis of alcoholism with convalescence 
from delirium tremens would certainly seem to have been 
sufficient for the phenomena, and the suggestion of alcoholic 
neuritis only confirmed the picture. To be sure, one might 
expect a diminution or absence of deep reflexes; still, these re- 
flexes may be over-active in an irritative stage of the disease. 



PUZZLES AND ERRORS 235 

Naturally, however, the history of syphilis and the pupil- 
lary phenomena and ptosis, made the consideration of neuro- 
syphilis necessary. Both serum and fluid W. R.'s proved 
positive; there was an excessive amount of albumin and 
globulin, the gold sol reaction was typically " paretic," and 
there were 377 cells per cmm. 

The patient improved upon a rest treatment and was 
given injections of mercury for his syphilis. After a few 
months he felt well enough to return to work, and continued 
at work throughout a season, receiving mercurial treatment 
throughout this time. A spinal fluid examination fifteen 
months later showed a weaker gold sol reaction, reduction 
in the amount of globulin and albumin, and but 26 cells to 
the cmm. The W. R.'s had remained positive. 

1. What are the forms of syphilitic neuritis? According to 

Nonne, syphilitic neuritis and polyneuritis have at 
last acquired standing in neuropathology. The older 
claims depended upon findings on palpation and re- 
covery after antisyphilitic treatment. Since the in- 
troduction of salvarsan, cases of ophthalmoplegia, 
facial, acoustic, and optic nerve disease, as well as 
neuritis of the extremities, have been reported in large 
numbers. These phenomena are to be regarded as 
neurorecidives in the modern sense of that term. The 
neurorecidive is not a salvarsan effect, but is an effect 
of the syphilitic process itself, settling in the peripheral 
nerves. Paresthesias are especially prominent in periph- 
eral mono- or polyneuritis, and this point is of some 
value in differentiating the syphilitic peripheral neuritis 
from root neuritis. Root neuritis is more often charac- 
terized by neuralgic attacks. Objective hyperesthesia 
of neuromuscular origin is also found in these cases, 
demonstrated by pressure on the nerves. The motor 
phenomena consist in a flaccid paresis or paralysis, 
especially affecting the radial, ulnar, and peroneal 
nerves. Nonne states that it is rare for syphilis to 
affect a single nerve region, and he regards cases in 
which a single region alone is affected as usually due to 
a local gummatous process. 

2. What is the significance of 377 cells per cmm.? See 

discussion of Washington (Case 66). 



236 PUZZLES AND ERRORS 



Differential diagnosis between NEUROSYPHILIS 
and CHRONIC ALCOHOLISM, 



Case 63. Albert Fielding, 46, was an insurance broker, 
who was brought to the hospital for excessive alcoholism. 
Indeed, he showed all the signs, both of chronic and acute 
intoxication, except that there was no nerve trunk tenderness. 
Fielding was very loquacious though his speech was rather 
thick. He showed tremor of hands and an alcoholic skin. 
Physical and neurological examination proved entirely neg- 
ative. 

Fielding claimed that he had had a nervous breakdown at 
about 36 years of age, after disappointment in love. He had 
the drinking habit and began to drink more and more. He 
had now become nervous and tremulous and had to drink in 
order to brace himself. After a few days, the patient began 
to be much better, having recovered from acute alcoholism. 
Mental examination now showed good memory with orienta- 
tion intact. There was a certain tendency to reminiscence 
and to somewhat childish actions. He had attempted to stop 
drinking but had been unable to quit. As a matter of fact, 
his mother and father had been excessive drinkers and he had 
inherited the tendency, etc. 

The diagnosis seemed to be plain. The routine W. R. 
upon the blood serum was negative. However, the patient 
had remarked during the history taking, that he had had a 
chancre and secondary symptoms of syphilis. Accordingly, 
lumbar puncture was resorted to. The fluid showed a slightly 
positive W. R.; the gold sol reaction was of the syphilitic 
type; there was a considerable increase in albumin and 
globulin, and there were 20 cells per cmm. The diagnosis 
of neurosyphilis seemed clear. 

Course : The patient received six months' treatment in a 
sanatorium but the symptoms remained almost as before, 
and the patient showed the same childishness and inability 
to take care of himself. Since the symptoms continued six 



PUZZLES AND ERRORS 237 

months after the withdrawal of alcohol, it might well be 
suspected that the condition was more than a merely alco- 
holic one. However, in a number of purely alcoholic cases, 
such long-standing effects are found: even as long as six 
months or longer after the withdrawal of the alcohol, and one 
might conclude therefore that Fielding was actually a victim 
of alcoholic dementia. The spinal fluid after these six months 
(during which period antisyphilitic treatment was given) 
showed no change, and the prognosis was offered that the 
case would probably develop into one of paresis. 

A year later, after six months sanatorial care and six 
months life in the community, the patient returned to the 
Psychopathic Hospital in an alcoholic condition. The lumbar 
puncture showed all signs negative except the W. R. which 
was slightly positive. The W. R. of the blood was negative. 

In connection with this case, see the case of paresis sine 
paresi (25). 

I. What is the relation of the syphilitic and alcoholic process 
in Robert Fielding? One does not like to break the 
so-called rule of parsimony in diagnosis, but it would 
seem that the effects in Fielding are the combined 
effects of syphilis and alcoholism. 



238 PUZZLES AND ERRORS 



Differential diagnosis between NEUROSYPHILIS, 
DIABETIC PSEUDOPARESIS and BRAIN 
TUMOR. 



Case 64. A large and imposing person, Calvin Hall, 55, 
had been employed as a doorkeeper and guard, in which 
position he was on duty for 12 to 14 hours daily. Eventually, 
however, he had begun to have a good deal of pain in the legs 
and a few months before observation, one day, his legs gave 
way and he fell to the floor. There was, however, no loss of 
consciousness, and he was carried to a general hospital. The 
result of an examination there was that his family was in- 
formed that he had some nervous trouble. 

Hall now began to be melancholy and wept a good deal. 
His appetite and sleep remained intact. He felt too weak to 
walk. At the end of about a year, he began to improve and 
again became able to do a little light work. About a month 
before coming to the Psychopathic Hospital, about two years 
after the onset of symptoms, Hall suddenly began to talk 
excessively, in a rambling and rather senseless way. A fort- 
night later, he began to suffer from insomnia and restlessness. 

Some medical facts were available: It seems that at 25 
years this patient had become infected with syphilis though 
there had never been any secondary signs. He was married 
four years later but there had not been any children. More- 
over, for four years past, the patient had been treated for 
glycosuria. 

Upon admission, the patient's sensorium was clear, but 
his orientation was only partial. He could give a fair account 
of his life, but it appeared that his memory was somewhat 
impaired. There were auditory hallucinations (voices of 
relatives). He often mistook the identity of persons about 
him. He talked in a grandiose fashion of his great strength 
and especially of a God-given power to read minds. His 
flow of thought was rapid, rambling, circumstantial, and with 
traces of irrelevance. He was rather continuously busy and 



PUZZLES AND ERRORS 239 

at times restive. There was a good deal of emotional agita- 
tion and apprehensiveness, and again the patient would 
become suspicious and tearful. 

Physically, there was a discharging sinus connected with 
the right humerus, close to the elbow. The pupils, though 
equal and regular, were sluggish in reaction to light. The 
knee-jerks and ankle- jerks were absent. There was no Rom- 
berg sign but there was some swaying in the Romberg position. 
There was a moderate ataxia in walking. Glycosuria to a 
moderate degree was determined. There were no casts or 
albumin in the urine. The W. R. of the blood and of the 
spinal fluid was negative. The albumin of the fluid, however, 
was considerably increased. X-ray examination of the skull 
yielded a suggestion of absorption of the posterior clinoid 
processes of the sella turcica. The X-ray examination of 
the arm in the region of the sinus showed a chronic os- 
teomyelitis, possibly syphilitic (or diabetic?). 

The diagnostic problems in the case of Calvin Hall are 
extremely intricate. There are clinical suggestions of general 
paresis, not confirmed by the laboratory findings. 

1. Are we dealing with a case of diabetic pseudoparesis? 

Is the pain in the legs of like origin, and has a neuritic 
process led to the absence of the knee-jerks? The 
Allen treatment appears to have had no beneficial 
result in this case. 

2. Is there a tumor of the sella region, which could account 

for the mental symptoms and the glycosuria? The 
spinal fluid albumin might be regarded as consistent 
with a variety of psychoses, including that of brain 
tumor. We have to remember the definite history of 
infection, the sterile marriage, and i.the. possibly syphi- 
litic osteomyelitis. 



240 PUZZLES AND ERRORS 



DIABETES AND NEUROSYPHILIS, relations? 



Case 65. Donald Barrie, a man of 61, diabetic for several 
years, had begun to worry about the diabetes, feeling that 
he was about to die, and had gone so far as to make several 
threats of suicide. Hence he was brought to the Psycho- 
pathic Hospital for observation. 

Barrie was rather well developed and nourished, although 
he looked far older than he was. There was a marked arcus 
senilis; the skin was dry and rough; the radial and other 
accessible vessels were markedly sclerosed; abdomen obese; 
right testicle very low with thickened and hard epididymis. 

Neurologically there was little abnormal to discover. The 
pupils were Irregular; both reacted fairly well to light. 
There was a slight tremor of the extended hands, and still 
less of the tongue. The voice was slightly thick and the 
patient stumbled somewhat on test phrases. Urine: specific 
gravity, 1029; sugar; no acetone; no diacetic acid. Sugar 2 
to II grams for 24 hours on ordinary diet. It proved im- 
possible to get the patient sugar-free, either by cutting down 
the carbohydrates or by using the Allen method. 

Mentally, the depression with reiteration of wrong-doing 
and self-accusation because of the contraction of syphilis, 
were the striking features. There was, to be sure, a slight 
imperfection of memory for remote events ; memory for recent 
events and knowledge of current events was very poor. 
Barrie claimed that his condition was entirely hopeless, that 
his memory was exceedingly bad, and that he was no longer 
capable of supporting his family. 

I. What shall be said as to diagnosis In a man of 61 with 
glycosuria, depression, amnesia, sluggish pupil, slight 
tremor, slight speech defect, and a history of syphilis? 
The W. R, of the serum proved positive, and also the 
W. R. of the spinal fluid. The gold sol reaction of the 
fluid was of the syphilitic type. There were 112 cells 



PUZZLES AND ERRORS 24 1 

per cmm., there was an excess of albumin, and a large 
amount of globulin. Accordingly, the diagnosis of 
PARETIC NEUROSYPHILIS (" general paresis "), espe- 
cially in view of the laboratory findings, seems necessary. 

2. What is the cause of the glycosuria? First: possibly 

it has no relation with the syphihs; secondly: it may 
possibly be due to a syphilitic involvement of the pan- 
creas; thirdly: it is barely possible that it is due to 
syphilitic disease of the fourth ventricle or of the base 
of the brain, involving the pituitary region. Perhaps 
our case is too complex for analysis. At all events, the 
case brings up the possibility of a syphilitic glycosuria. 

3. Can the diabetes in the case of Barrie be explained as 

syphilitic? Warthin of Ann Arbor has recently de- 
scribed somewhat remarkable spirochete findings in 
his autopsy material. The order of organic infection 
according to frequency is: aorta, heart, testis, adrenal, 
pancreas, nervous system, liver, and spleen. Warthin 
has called attention to the relation of pancreatitis and 
spirochetosis to diabetes in a recent review * of findings 
in 41 autopsied cases from the University Hospital in 
Michigan. Warthin found active luetic lesions in the 
pancreas in 6 cases. 

* Warthin: " Persistence of active lesions and spirochetes 
in the tissues of clinically inactive or * cured ' syphilitics,'* 
American Journal of Medical Sciences, CLII, 191 6. 



242 PUZZLES AND ERRORS 



Hemianopsia in a case of neurosyphilis. 



Case 66. Lawrence Washington, a colored cabman, 58 
years of age, began to forget addresses given him by his fares. 
Moreover, he could no longer see as well as before, especially 
on looking toward the right side. He himself states that the 
visual trouble dated back as long ago as his 39th year, at 
which time he had a terrific pain in both temples, leading 
back from the eyes. Washington thought that his vision 
had been getting slowly but steadily worse ever since. 

We got the impression that the amnesia claimed by Wash- 
ington was more or less subjective and he was found to be 
well informed. This association of amnesia and impairment 
of vision naturally suggests syphilis. The patient himself 
stated that he had had a chancre at the age of 18. 

We found the W. R. of the serum to be appropriately pos- 
itive. The W. R. of the spinal fluid was also positive though 
weakly so. There was an excess of albumin; globulin ap- 
peared In large amount ; the gold sol reaction was of the syph- 
ilitic type; there were 186 cells in the spinal fluid. 

Is this case one of paresis or of some other form of cerebro- 
spinal syphilis? Let us consider the data of the physical 
examination. On the whole, the patient was well preserved. 
There was a slight radial arteriosclerosis, but on the whole 
the cardiovascular system was almost negative. The blood 
pressure was 100 systolic, 65 diastolic. Neurologically the 
visual field of the left eye was somewhat limited, and there 
was a temporal hemianopsia of the right eye. The ophthal- 
moscopic examination showed a disseminated choroiditis on 
both sides. The right pupil failed to react to light. The left 
pupil reacted slowly. Both pupils reacted properly to accom- 
modation. 

The knee-jerks could be obtained only on reinforcement, 
and when obtained, the right was apparently more active 
than the left. The left Achilles was absent ; the right present. 
There were no other abnormal reflexes. 



PUZZLES AND ERRORS 243 

The motility of the facial muscles was somewhat impaired. 
Finger- to- finger and finger-to-nose tests were rather poorly 
done. The muscle sense was good; there was no swaying 
in Romberg position ; and there was no speech defect. 

We are unable to decide whether the case is one of the 
parenchymatous type (paretic) or of the meningovascular 
type of neurosjrphilis. It is certainly rather unusual to find 
hemianopsia in a paretic. 

We have been unable to get definite results from the treat- 
ment of this case, since the patient would not return for 
months after getting an injection or two of salvarsan, on the 
ground that he was improved enough and did not require 
further treatment. 

I . What conclusion can be drawn from the 1 86 cells per cmm. 
in the spinal fluid? Ordinarily this finding would 
indicate an active process. Some writers have claimed 
that a cell count running above lOO per cmm. was an 
indicator of diffuse non-paretic neurosyphilis. It does 
not appear that this claim has been substantiated. 
It is remarkable that this case shows an Interval of 40 
years between infection and the occurrence of definite 
clinical symptoms. With respect to the cell count, 
both in untreated and in treated cases, the following 
conclusions from a recent article (Solomon and Koefod)* 
are In point: 

1. The number of cells found in the fluid of un- 
treated cases offers no definite information of prog- 
nostic value. 

2. One is not justified in drawing any conclu- 
sions as to whether the case is cerebrospinal syphilis 
or general paresis, nor the time the process has been 
active, nor the severity of it, from the cell count. 

3. The cell count may vary greatly from month to 
month, or when the Interval Is but several days, while 
at other times it may remain very nearly the same 
after an interval of months. 

4. Cases showing natural remissions may show no 
reduction in the cell count, or other spinal fluid findings. 

* ** The Significance of Changes In Cellular Content of 
Cerebrospinal Fluid In Neurosyphilis," Boston Medical and 
Surgical Journal, CLXXIII, 27. 



244 PUZZLES AND ERRORS 

5. Cases treated with salvarsan, ^either intraspi- 
nously or intravenously, tend to show a more or less 
rapid fall in the cell count. This count will, as a rule, 
remain low during treatment, but is likely to rise when 
treatment has been discontinued, but may rise during 
treatment after having first fallen. 

6. Cases may show remissions during treatment 
and still have a pleocytosis. 

7. Treated cases having the cell count fall to normal 
may at the same time become very much worse and 
develop more marked paralytic symptoms. 

8. In general paresis the cell count in no way paral- 
lels the other spinal fluid findings. 

9. In cases in which the other tests show an im- 
provement, for instance cerebrospinal syphilis, the cell 
count also readily and early drops to normal. At times 
it may drop to normal before other spinal fluid tests 
become negative; again it may be last to reach normal. 

10. The change in cell count seen in syphilitic 
disease untreated is also found in non-syphilitic diseases, 
as brain tumor. 

11. The cell count offers nothing of prognostic 
importance in syphilis of the nervous system unless ac- 
companied by improvement of the other laboratory 
signs. 

12. The cell count is not an index to the predomi- 
nance of irritative or degenerative changes. 



PUZZLES AND ERRORS 245 



Case of CEREBRAL MALARIA and SYPHILIS 
simulation of P^ 
eral paresis "). 



Case 67. Joseph Temple, 45, who had been a sea-going 
steamboat steward, was brought to the hospital in a semi- 
stupor. He was entirely uncooperative, often resistive, at- 
tempting to bite the physician's fingers, and for the most 
part lying curled up. He was incontinent and tube-fed. 
This phase, it seems, had begun the night before entrance to 
the hospital. Twenty-four hours later, an extraordinary 
change was noted. Temple became alert and attended to his 
wants, began to eat well, and began to behave as normally 
as probably he ever behaved. 

He was now able to give a coherent history. It was now 
January. In the previous September, he had left for Mexico; 
he was returning when he suddenly fell to the deck, uncon- 
scious. After this fall, he had not been well, having had 
chills and fever. At the Marine Hospital, he had been 
diagnosed as suffering from malaria, and was given quinin. 
He had been delirious a short time in the hospital, not being 
able to recognize his wife, who called. He shortly improved 
so that his wife was able to take him home. Nevertheless, 
headache, gastric distress, and intermittent vomiting con- 
tinued. A spell of confusion took place, two days before 
admission. The patient tossed about, moaned, and failed 
to recognize anyone. Malaria of the sestivo-autumnal type 
was demonstrated in the hospital. The temperature always 
remained at normal. He was somewhat emaciated and pale. 
The pupils were small, somewhat unequal, and reacted though 
poorly to light and distance. The tendon reflexes were 
lively. 

The W. R. of the serum was positive, and information from 
the patient's physician runs to the effect that there was a 
syphilitic infection some seven or eight years ago, followed 
by secondary symptoms, but the patient had refused to take 



246 PUZZLES AND ERRORS 

any protracted treatment. The spinal fluid examination 
was practically negative. 

Mentally, the patient was euphoric, expansive, boastful, 
and showed a marked emotional instability and considerable 
memory defect. 

I. Can the diagnosis of general paresis be made in Joseph 
Temple? Certainly the acute confusion and the syncope 
are consistent enough with the diagnosis, yet the severe 
malaria makes it seem likely that the phenomena were 
due to a cerebral attack of malaria, and such occur- 
rences are found in the aestivo-autumnal form of ma- 
laria. Yet malaria would hardly explain the euphoria, 
memory defect, and the pupillary findings, to say noth- 
ing of the Irritability and the active tendon reflexes. 
Even if we regard the active tendon reflexes and the 
irritability as malarial, the other phenomena remain 
outstanding as exceedingly suspicious of paresis. 

On the other hand, if we try to support forcibly the 
diagnosis of general paresis, we are hardly able to ex- 
plain the negative findings in the spinal fluid. 

In point of fact, a study of the patient's past life 
revealed a story that the mental traits of euphoria, 
irritability, and memory defect had been characteristic 
of the patient for many years. In fact, there Is some 
question whether the patient is not really to be regarded 
as a moron of high grade. 

Upon this basis, If we regard the confusional phe- 
nomena as malarial and the persistent mental phenomena 
as characteristic of a moron and somewhat exaggerated 
by the disease, we have merely to explain the suggestive 
pupils. As to these. It must be remembered that though 
they reacted poorly to light, still they reacted somewhat, 
so It is not a question of explaining an Argyll- Robertson 
pupil, but only an impaired pupillary reaction. Of 
course, some workers are of the opinion that pupil- 
lary changes, perhaps even the Argyll- Robertson pupils, 
may occur In syphilitic cases that are not neurosyphilltic, 
or at all events are not victims of central neurosyphilis. 
Finally, we must remember that there are cases of 
neurosyphilis of a vascular type which yield negative 
spinal fluids. The case leaves many questions unan- 
swered. 



PUZZLES AND ERRORS 



247 



Can paretic and non-paretic neurosyphilis be dif- 
ferentiated by means of the gold sol reaction? 
The gold sol reaction in this case was an extremely 
mild one and would not at all have warranted the 
diagnosis GENERAL PARESIS, yet the discovery 
of a heavy meningeal exudate including an un- 
usually heavy deposit of plasma cells even in the 
spinal pia mater will perhaps warrant us in making 
a final retrospective diagnosis of paretic neuro- 
syphilis. Autopsy. 



Case 68. We would like to give the full effect of our sur- 
prise at the outcome of the case of Margaret O'Brien, a 
school-teacher, 26 years of age. To be sure, Miss O'Brien 
developed symptoms at 22 or 23 which we can now explain 
consistently with the outcome of the case; for at that time, 
she began to complain of severe pain in the head, especially 
in the forehead and temples, and also became nervous, unable 
to remain quiet, and given to insomnia. She was markedly 
depressed at the time and would refuse to talk at times. 
However, only the headache In this prodromal period could 
be regarded as particularly suggestive of syphilis, and head- 
ache in an over-worked school-teacher Is not uncommon. 

In fact, the picture presented by the patient was one of 
catatonic dementia praecox. The patient was admitted to 
the hospital after a sudden onset of excitement. At first she 
was very restless, continually looking about and getting up 
and walking away from the examiner, giving the Impression of 
understanding all questions but preserving an air of Indif- 
ference. A few days later, the patient was gotten to answer 
more cooperatively. She remarked that the hospital was 
heaven although In Boston; that It was summer time (correct) 
and that her memory was greatly impaired. The physician 
was a messenger of God (delusion later corrected). The 
patient had not done God's will; her breath was leaving her; 
God's voice was heard from time to time, and Miss O'Brien 



248 PUZZLES AND ERRORS 

had heard it for a long time. God tells her to do His will. 
However, as Miss O'Brien remarked, " I must think all this 
nonsense, turning against God." 

The patient frequently attitudinized and would remain 
in an apparently catatonic condition for many minutes. 
For the most part, she was resistive and mute and non- 
cooperative as to examination. From time to time, she 
made impulsive suicidal attempts. So far as a somewhat 
inadequate physical examination was concerned, nothing ab- 
normal could be made out; in particular, the pupils reacted 
normally to light and were otherwise normal. The routine 
W. R. of the blood serum, however, returned positive, and 
in accordance with the policy of the Psychopathic Hospital, 
the patient was subjected to a lumbar puncture. The lum- 
bar puncture yielded a positive W. R., 109 cells per cmm., a 
positive globulin and a considerable excess of albumin, and 
an exceedingly mild gold reaction — syphilitic type. 

Ten days after admission, the patient had a convulsion. 
She never regained consciousness, continued to have convul- 
sions for a few hours, and died, apparently from paralysis 
of respiration. The heart continued to beat for a short 
period after respiration ceased. The autopsy was consis- 
tent with the diagnosis which had been rendered after the 
surprising results of the W. R. In the blood and the laboratory 
findings in the spinal fluid had been learned. There was a 
generalized encephalitis with congestion of all the smaller 
cerebral vessels and petechial areas in the meninges and 
upon the cortical surfaces. We regard the case as one of 
syphilitic encephalitis. 

The brain weighed 1265 grams, indicating a loss of 79 
grams by TIgges' formula (8 times the body length in centi- 
metres). The pia mater was. In the gross, quite normal 
within the cranium; nor were any cells found in a smear 
from this pia mater; but the pia mater over the spinal cord 
was visibly edematous, and a smear from the spinal pia 
mater showed great numbers of lymphocytes and especially 
of plasma cells — a finding which was confirmed In stained 
section, by which a remarkable display of plasma cells was 
found plastered somewhat generally over the entire pia 



PUZZLES AND ERRORS 249 

mater of certain segments. The brain substance was softer 
than normal, but displayed no differences of consistence. 
The stripping of the pia mater of the temporal lobes on both 
sides yielded the so-called " decortication " (that is, the adhe- 
sion of small bits of brain substance to the pia mater). The 
optic nerves were somewhat thinner than normal. No other 
gross lesions of the brain were found. 

The dura mater, although dense and injected, was not 
otherwise abnormal. There was an early visible sclerosis 
of the middle meningeal arteries, more marked on the left 
side. 

The cause of death, so far as the autopsy revealed it, 
was bronchial pneumonia. There was a diffuse nephritis. 

1. Are the hallucinations in the case of O'Brien character- 

istic? Hallucinations are regarded as playing a minor 
r61e in general paresis. In fact, earlier workers some- 
times denied that hallucinations occurred at all, and 
this denial has been made once more of late by Plaut,* 
but Kraepelin quotes Obersteiner as observing hallu- 
cinations in 10%, and regards that figure as approx- 
imately corresponding with his own experience. Junius 
and Arndt are cited as finding 17% of their cases hallu- 
cinated. Auditory hallucinations are somewhat more 
frequent than those of vision (alcoholic psychosis must 
be considered). The visual hallucinations of paresis are 
thought by Kraepelin to be related with atrophy of the 
optic nerves, and he states that they occur by prefer- 
ence in patients having such atrophy. Hallucinations 
though not common are more frequent in non-paretic 
neurosyphilis than in paretic neurosyphilis. 

2. What was the cause of death in Margaret O'Brien? 

The autopsy, as above stated, indicated pneumonia. 
In point of fact, this patient developed convulsions and 
ceased respiration, the heart continuing to beat for 
some time after respiration had ceased. It may be 
that the death should be counted as one of neuro- 
syphilitic seizure. 

* Plaut: Ueber Halluzinosen der Syphilitiker, Berlin, 1913. 



250 PUZZLES AND ERRORS 



Tonsillar abscess associated with neurosyphilis 

(Lues Maligna?). 



Case 69. Frank Mason, 49 years, a rectifier of spirits, 
was admitted to the Psychopathic Hospital in a tremulous, 
mentally confused, depressed, and unhappy state. He was 
particularly concerned because he could not give an accurate 
account of his past life and because he found that he was 
continually contradicting himself. 

Superficial examination shortly discovered the pupils to be 
much contracted, irregular, and non-reactive either to light 
or distance. Although these pupils showed more than the 
Argyll- Robertson phenomenon, yet the suspicion of syphilis 
was important. 

Throat examination showed a large area of ulceration 
involving the whole of the right tonsil and extending even 
to the left side of the median line so that the whole of the 
faucial pillar was involved. In the midst of this ulcerative 
area was a mass of purulent necrotic tissue, about which the 
edges of the ulcer stood out sharply. There was, however, 
very little acute reaction about the margin of the area. 

The association of pupillary changes (especially stiffness 
to light), what looked like tonsillar gumma, and mental dis- 
order (including memory disturbance) heightened the impres- 
sion of syphilis. 

However, the remainder of the examination was not es- 
pecially confirmatory of the diagnosis. The man was well 
developed and obese, with a slightly enlarged heart, with 
sounds of poor quality and the aortic second sound accen- 
tuated. The systolic blood pressure was 130; the diastolic, 
90. There was no disorder of reflexes except that the arm 
reflexes were very lively. 

After a time, a few facts concerning the patient's life 
became available. Although a rectifier of spirits. Mason 
could not be found to have over-indulged in alcohol. It 
appears that some five months before his admission to the 



PUZZLES AND ERRORS 25I 

hospital, a wisdom-tooth had been extracted. About four 
months before admission, the ulceration of the faucial pillar 
had begun, and this ulceration was immediately, laid to in- 
fection from the wisdom-tooth cavity. Mason then had to 
discontinue work and a depression followed. But the account 
of this depression led us to think that he was a victim more 
of natural sadness than psychopathic depression. There 
was much worry and insomnia. To meet the insomnia, 
large amounts of hypnotics were administered. The sequence 
of these hypnotics was a tremendous disturbance and contin- 
ual crying out by the patient. In fact. Mason became so 
excited that he was removed to the Psychopathic Hospital 
for temporary care in the condition above mentioned. 

We naturally awaited the outcome of the serum W. R. 
The return was negative. However, the typical position of 
the ulcerative lesion and the non-reacting pupils, — to say 
nothing of the mental symptoms and the associated tremors, 
with incoordination (this incoordination was non-characteris- 
tic and apparently due largely to the tremor), — led to lumbar 
puncture. 

The spinal fluid yielded a weakly positive W. R. There 
was a slight positive albumin, the globulin test was slightly 
positive, there were 14 cells per cmm., and the gold sol reac- 
tion was of the syphilitic type. We were, then, probably 
entitled to conclude that syphilis was active not only in the 
body at large but also in the nervous system. Looking back 
upon the case, we considered that large doses of morphine and 
hyoscyamus might well have produced the marked mental 
confusion and possibly the tremors that characterized Mason 
on his arrival at the hospital. 

Improvement followed after a few days of rest; the con- 
fusion disappeared and the tremors diminished; the pupils 
returned to their normal size and reaction; depression per- 
sisted, and the patient was very properly much concerned 
about the tonsillar lesion. However, further improvement 
did not take place under antisyphilltic treatment and patient 
died after several weeks from what was believed to be an 
embolus from the tonsil. 



252 PUZZLES AND ERRORS 

1. What was the true interpretation of Frank Mason's 

pupillary changes? They were probably due to the 
opiates, despite the fact that, taken in association with 
the gummatous lesion of the faucial pillar, we had re- 
garded them as possibly syphilitic. 

2. How shall the negative serum W. R. be explained? 

Such a reaction is consistent with the diagnosis gumma. 
It is, however, a little surprising that with active neuro- 
syphilis and a relatively active non-nervous syphilitic 
lesion like that in this case, the serum W. R. should 
have been negative. Possibly a repetition of the test 
at various times would have shown a positive serum 
W. R. In any event, the fluid reaction was positive. 

3. Could the tonsillar ulceration be due to dental infection? 

The chances are against this on account of the interval 
(2 months) between extraction of the wisdom tooth 
and the ulceration, which itself seems to be of a ter- 
tiary syphilitic nature. In point of fact, the patient 
admitted a syphilitic infection 2T years previously 
namely, at 28 years of age. At that time he took 
large quantities of mercury and potassium iodid by 
mouth. 

4. Relation of the case of Frank Mason to the so-called 

lues maligna? The case closely resembled the cases 
reported by Bly. Frank Mason showed great destruc- 
tion of tissue, toxemia, failure to react to antisyphilitic 
treatment. In both of Ely's cases, the tonsil was the 
starting point of the illness ; and in both cases there was 
a trauma of the tonsil or peri-tonsillar structures (ton- 
sillectomy and application of caustic). In our case 
there not only had been extraction of a wisdom tooth, 
but the tonsil had been cauterized. 



PUZZLES AND ERRORS 253 



Neurosyphilis versus multiple sclerosis. 



Case 70. Annie Kelly is a young Irish woman, 21 years of 
age, who was perfectly well until three months before her 
admission to the Psychopathic Hospital, when suddenly 
one evening she became very dizzy. This was followed by a 
chill and vomiting. The next day she had a sore throat but 
was able to be about and do her work. The dizziness, 
however, continued and she began to feel rather queer. 
Gradually it became difhcult for her to walk on account of 
staggering. 

A little later she noticed a weakness of the left side, in- 
volving face, arm, and leg; then she began to find it diffi- 
cult to talk. Finally the right leg became weak, making 
walking practically Impossible. All these symptoms grew 
worse and the dizziness increased. At times her vision would 
be blurred ; there were somewhat frequent attacks of diplopia. 
Finally she had to take to her bed, and at last she lost con- 
trol of her sphincters. 

At no time did she suffer any pain. She was taken to a 
hospital, and after a time improved somewhat; but she was 
told she had a brain tumor and had better be in a large city, 
where she could have surgical aid if this became necessary; 
consequently, she was brought from Montana to Boston. 

On admission to the hospital, the examination disclosed no 
important symptoms outside of the nervous and locomotor 
systems. She was unable to walk unless assisted. The 
pupils were large but reacted well to both light and accommo- 
dation, were equal in size, and regular. Slight nystagmus 
was present; there was no ptosis or strabismus; vision in 
the left eye was poor. The other cranial nerves showed 
no involvement. The tendon reflexes were all present and 
very lively; Babinski, Gordon, and Oppenhelm signs were 
present on either side. The ataxia was marked, especially 
of the lower arms, and she had some difficulty In the align- 
ment of the fingers. The sense of position of the limbs was 



254 PUZZLES AND ERRORS 

very poor. There was some tremor, which was not of the 
intention type. The writing showed some incoordination. 
The speech showed nothing abnormal. Mental examination 
disclosed nothing of note objectively, but patient stated she 
could not think so clearly as she could formerly. 

The diagnosis would seem to lie between brain tumor, — 
which had been suggested to the patient by her physician, — 
multiple sclerosis, and neurosyphilis. The numerous neuro- 
logical symptoms without any definite evidence of intra- 
cranial pressure were sufficient to rule out for the moment 
the consideration of brain tumor. The syndrome of multiple 
sclerosis is not complete, but the race, age, and onset, with 
the increasing and decreasing intensity of symptoms are 
very suggestive of this diagnosis. The symptoms, of course, 
are all consistent with neurosyphilis also, and while the pa- 
tient denied any knowledge of syphilitic involvement, the 
examination of the blood and spinal fluid was made. The 
W. R. was negative in both the blood serum and spinal fluid. 
Further examination of the spinal fluid showed presence of 
globulin and an increase in the albumin content, 43 cells 
per cmm. and a " paretic " type of gold sol reaction. With 
the negative W. R. of both blood serum and spinal fluid, 
and with so much in favor of Multiple Sclerosis, this 
diagnosis was made. 



What is the relation of multiple sclerosis to syphilis? 
There is no definite relationship between multiple 
sclerosis and syphilis, — that is, multiple sclerosis is 
not a syphilitic disease; but the complete syndrome 
of multiple sclerosis is often given by a syphilitic in- 
volvement of the central nervous system (see case 
Lauder, 71). 

Is the spinal fluid finding in this case consistent with 
multiple sclerosis? According to Nonne, about 19% 
of the cases of multiple sclerosis show globulin and 
pleocytosis in the spinal fluid. As a rule, the number of 
cells ranges between 10 and 20 per cmm. and the glob- 
ulin is not present in large amounts. In this case, the 
amount of globulin, which was given as 2-|-, is only a 
moderate amount, — less than is usually found in 
cases of general paresis. There are not very many 



PUZZLES AND ERRORS 255 

cases of multiple sclerosis In the literature in which 
a gold sol reaction has been performed, but in the 
majority of those tested, the reaction is reported as 
mild. However, cases of multiple sclerosis giving a 
typical paretic curve have been described by a number 
of observers, among whom may be mentioned Kaplan 
and Solomon. 
3. How frequently is it necessary to make a differential 
diagnosis between multiple sclerosis and neurosyphilis? 
Before the days of the W. R. this differentiation was 
much more difficult than at present. But we, however, 
still have to face a not very rare difficulty in separating 
the two conditions. Syphilis Is prone to cause small 
localized lesions In the nervous system. The changes 
in the patient's condition, with improvements and 
regressions are equally characteristic of both diseases. 
How closely the symptomatology of neurosyphilis may 
simulate that of typical multiple sclerosis Is shown in 
the next case (Lauder, 71). When the sclerotic area 
of multiple sclerosis occurs in appropriate parts of 
the cerebrum, symptoms of mental disturbances will 
occur. In Its histological picture multiple sclerosis Is 
at times highly suggestive of syphilis, even showing 
mononucleosis and meningitis. 



256 PUZZLES AND ERRORS 



Optic atrophy; nystagmus; spasticity; intention 
tremor. Diagnosis : ? 



Case 71. James Lauder began to lose his eyesight at 32 
years, and was shortly determined to be suffering from 
primary optic atrophy. In the course of a year, he had 
become completely blind. No mental symptoms had de- 
veloped. 

Physically, Lauder was In very good condition. Neuro- 
logically, there was a complete optic atrophy with paralysis 
of the internal rectus muscle, marked nystagmus, and ab- 
sent pupillary reactions. All the tendon reflexes were ex- 
ceedingly lively, though the right arm reflexes were more 
lively than the left, and the left leg reflexes more lively than 
the right. There was an ankle clonus on both sides. The 
abdominal and cremasteric reflexes were lively. There was 
a slight intention tremor. There was, however, no ataxia 
and no speech defect. 

Diagnosis: The nystagmus, optic atrophy, and the re- 
flex disorder suggested multiple sclerosis, although the 
liveliness of the superficial reflexes, especially the abdominal 
reflexes, was a point somewhat against any advanced degree 
of multiple sclerosis. It would appear that the absence of 
pupillary reaction to accommodation Is also rather unusual 
in multiple sclerosis. 

The serum and spinal fluid W. R.'s proved positive. There 
, were 25 cells per cmm., albumin was in excess, and there was 

a positive globulin reaction. 

M I. What Is the significance of optic atrophy and other 

optic changes with respect to neurosyphilis? Canavan, 
from our laboratory, has reported that she found that 
40 of 58 unselected cases of mental disease exhibited 
obvious and undeniably important changes in the 
optic nerve. She found that optic nerve changes were 
even more frequent than chronic spinal cord changes 
as detectable by the same method (Weigert myelin 



PUZZLES AND ERRORS 257 

sheath method); there were only 34 of the 58 cases 
which showed chronic spinal cord changes. Eighteen 
cases very probably syphilitic (although the clinical 
evidence was not in all cases supported by the W. R.) 
failed to show optic nerve changes in but three in- 
stances. The 15 syphilitic cases that did show optic 
nerve changes showed them in but one eye in three cases, 
in both eyes in 12 cases. Canavan incidentally dem- 
onstrated a spirochetosis in the pial sheath of the optic 
nerve in a case of neurosyphilis, possibly paretic. 
What is the frequency of eye changes in neurosyphilis? 
Posey and Spiller (" The Eye and the Nervous System," 
1906) quote Keraval as finding 42 instances of fundus 
change in 51 cases of paresis. Clifford Allbutt found 
41 cases of atrophy in 53 of paresis ; other authors have 
found far fewer. Optic atrophy sufficiently marked to 
cause blindness is relatively rare in paresis. Com- 
pare table of eye changes from Joffroy under Case 
Falvey(55). 

As for optic atrophy in tabes, Posey and Spiller 
record statistics as so various as to be on the whole 
unsatisfactory. The highest percentages found appear 
to be those of Mott, 80%, and Gross, 88%. It is evi- 
dent that the standards for measuring optic atrophy 
must differ very much. 



258 PUZZLES AND ERRORS 



Atypical case of neurosyphilis. Picture of Hunt- 
ington's chorea. 



Case 72. Margaret Green, 28, was received at Danvers 
State Hospital in an excited and frightened state. She was 
very talkative and said that she was being bitten by snakes 
and serpents. She thought every one approaching her was 
the devil, and sprinkled what she called " holy water " about 
her for protection. It was clear that she was hallucinated. 
She heard her child crying, and she saw a woman carrying it 
away. 

After a few weeks, Mrs. Green grew quiet and more rational 
except for a few spells of violence and noise; she gave the 
impression of a rather pleasant and agreeable, though some- 
what demented, patient. Physically, beyond a tremor of 
fingers and tongue and lively knee-jerks and some evidence 
of enlargement of the heart, there was nothing to be found. 

Margaret Green is still in the Danvers Hospital, being now 
48 years of age. During the twenty years, she has presented, 
— besides the mental picture of impairment of memory — 
occasional spells of confusion, a variety of delusions based, 
at least in part, upon auditory and vivid visual hallucinations, 
a certain irritability and psychomotor excitement, and a 
picture of Huntington's chorea. The diagnosis of Hunting- 
ton's chorea has always been in doubt by reason of the lack 
of any evidences of hereditary taint; it has, however, not 
been possible to secure a properly intensive account of her 
relatives. 

It appears that the choreic movements were first ob- 
served — in the hospital at least — about 16 years ago. The 
patient has always been decidedly mixed upon dates. From 
internal evidence derived from her obviously in part erroneous 
statements, it may be that the chorea began at the age of 
23. It appears that she had been often termed a victim of 
of St. Vitus' dance, and had had to leave her work In the mill 
on account of the disease. From one source of information, 



PUZZLES AND ERRORS 259 

it would appear that the patient began to have what was 
called St. Vitus' dance when she was 14 or 15 years of age; 
so far as this informant knew, no other member of the family 
had had the affliction. 

The first movements observed in the hospital were irregular, 
jerking movements, more marked in the left arm but also 
occurring in the other extremities, as well as in the face, 
wherein were produced peculiar grimaces. The twitching 
movements would become decidedly worse during spells of 
irritability. Observation in the patient's early thirties left 
the question in doubt whether the left pupil reacted to 
light or not. In 1904, when the patient was 36, both pupils 
failed to react to light either directly or consensually. At 
this time, the jerky movements continued, especially in the 
left hand and forearm, the tongue was tremulous, test 
phrases were poorly pronounced, the knee-jerks were exag- 
gerated (especially the left), and both wrist- jerks were exag- 
gerated. The systematic examination, however, revealed 
no other neurological disorder. Within a year, slight spuri- 
ous ankle clonus developed on both sides; the eyes, es- 
pecially the left, gave the appearance of developing cata- 
racts. A slight consensual light reaction was demonstrable 
on the right side, but all light reactions were absent in the 
left eye. 

At the age of 42, the patient was still disoriented for time, 
place, and persons and subject to a deep amnesia; was 
tidy, tranquil, and of a pleasant demeanor, but many of her 
muscles were in continual motion. There were chewing 
movements and both hands and feet were rarely still. There 
were no longer any spells of irritability or violence; and 
once when found crying on the piazza, Mrs. Green, on being 
asked the reason, replied that a gray cat had come and looked 
at her so hard it made her cry. There were other crying 
spells at times for equally good reasons, or for no reason. 

More recently, the patient has become fairly well oriented 
for time and place, and has acquired a fairly good insight 
into her condition and a good memory for past events. 
She has had occasionally auditory hallucinations, as of water 
running. In 1914, it was reported that the pupils reacted 



260 PUZZLES AND ERRORS 

to light, and the rest of the systematic neurological examina- 
tion was negative except that the knee-jerks were exaggerated ; 
and a re-examination in 191 6 showed the pupils still reacted to 
light. At present, the patient is disoriented for time, stating 
that her age is about 25; she is no longer subject to auditory- 
hallucinations; she has a marked difficulty in enunciation, 
emphasized by the lack of teeth and in part due to continual 
movements of the tongue; the movements appear to be part 
of a generalized chorea. 

In a systematic review of the Wassermann findings in the 
hospital population, the blood of Margaret Green was ex- 
amined and found to be positive. Lumbar puncture forth- 
with performed showed a positive W. R. in the fluid; there 
was a positive globulin and an excess of albumin; the gold 
sol was characteristic of paresis; there were, however, but 
three cells per cmm. 

I. Are the choreiform movements related to the demon- 
strable syphilis of the nervous system? Nziither the 
fluid W. R. nor the gold sol reaction should be regarded 
as necessarily an indicator of tissue loss. The fluid 
W. R. is commonly thought to signify merely that the 
fluid contains substances which are somehow due to the 
presence of spirochetes in some region pretty closely 
related with the fluid. The gold sol reaction, although 
well established to be characteristic of neurosyphilis, 
is perhaps not so strong an evidence of the existence of 
spirochetes in the region from which fluid constituents 
are derived. There is no pleocytosls. However, the 
positive globulin test and the excess of albumin do 
indicate a certain amount of destructive process some- 
where in the neural tissues. Are we to suppose that 
these substances have been continually found during 
the course of this disease? This question cannot be 
answered with the data in hand, and we can only sus- 
pect that these positive tests for albumin and globulin 
are an effect of tissue destruction caused by neuro- 
syphilis. It must be admitted that the argument 
here is a little tenuous. The lesson is plain: that 
in the present stage of our knowledge the W. R. 
should not be omitted even in cases which present a 
fairly convincing picture of some well-known entity. 
Thus, a disease, which looks like Huntington's chorea, 



PUZZLES AND ERRORS 26l 

as well as a disease suggestive of multiple sclerosis, 
requires investigation by the methods of the syphi- 
lographer. 

2. How shall we explain the changes in pupillary reaction 

in this case? They cannot yet be explained. A few 
observers have reported changes in pupillary reflexes 
in the direction of normality. In our experience such 
changes have not been noted. It cannot be too strongly 
emphasized that it is very easy to make errors in 
judging pupillary reaction if care Is not used. For 
instance, if the patient is accommodating for near 
vision, light will probably not cause contraction. A 
frequent cause of error in testing the light reflex arises 
from using a weak electric light. An electric flash- 
light is much less efficient than daylight. Probably 
the most satisfactory method is to take the patient to 
a window, ask him to look at a distant object, shade 
the eye with the hand, remove hand, and observe. 

3. What is the chief triad of symptoms in Huntington's 

chorea? (i) Choreiform movements associated with 
(2) progressive mental enfeeblement, (3) occurring in 
a patient whose family history shows a similar con- 
dition in a preceding generation. 



262 PUZZLES AND ERRORS 



Differential diagnosis between NEUROSYPHILIS 
and SENILE ARTERIOSCLEROTIC PSYCHOSIS. 



Case 73. Marcus Chatterton was a retired sea captain, 
75 years of age. At the age of 71, he had had a seizure with 
a sHght right hemiplegia and inability to talk. He had been 
slightly confused for a short time but had rapidly recovered. 
During the intervening four years, there had been three 
similar attacks, and the last one had caused him to come to 
the hospital. He was, in fact, confused upon admission 
but had become perfectly clear by the next day. There was 
a considerable memory defect, which the patient himself 
did not entirely appreciate. Possibly his judgment had been 
deteriorating slightly. He had been irritable of late and 
sometimes sleepless. 

Physical examination showed a rather well-preserved 
man with but slight senile changes. The pupils were equal 
and reacted readily to light and accommodation. There 
was no sensory disorder and no disturbance of coordination. 
There were no tremors. The systolic blood pressure was 205, 
the diastolic 135. The arteries were sclerotic upon palpation. 
A sufficient diagnosis would have seemed to be arteriosclerosis, 
and the hypothesis of syphilis would hardly have been raised 
off-hand by most practitioners. The W. R. of the serum was 
negative. What led to lumbar puncture in this case was the 
fact that the sea captain's wife had died 15 years before of 
general paresis. The lumbar puncture was rewarding since 
the W. R. was positive. There was an increase of albumin 
and globulin, a " paretic " type of gold sol reaction, and 56 
cells per cmm. 

Accordingly, we must regard the condition as one of 
neurosyphihs. Perhaps the arteriosclerosis was of syphilitic 
origin. If this is a case of general paresis as we suppose, 
it is one of very long-standing syphilis. 



PUZZLES AND ERRORS 263 

1. Do delusions of grandeur in the senile period suggest 

syphilis? Not necessarily; it appears that there is a 
small group of senile cases which might be called cases 
of senile pseudoparesis in which extravagant delusions 
of grandeur are entertained, and in which frontal 
atrophy is found although entirely without evidence of 
chronic inflammation. It has not been proved that 
these cases are of syphilitic origin. It is suggestive 
that the site of the most extensive lesion is precisely 
the site of the most extensive lesion classically found in 
paretic neurosyphilis, viz., in the frontal regions. 

2. Is neurosyphilis frequently found in both mates? It 

can hardly be said that this is a usual finding. How- 
ever, it is far from rare, and it occurs frequently enough 
to be used in support of the theory that there is a 
special strain of spirochete that has a predilection for 
nervous tissue. It must be remembered, however, 
that the wives of syphilitics are frequently infected 
without being aware of it. In such cases they re- 
ceive no treatment and consequently have a larger 
chance of developing neurosyphilis. It is a good rule 
to consider the mate of every syphilitic a candidate for 
neurosyphilis. J 



I 



264 PUZZLES AND ERRORS 



An atypical case of recurrent dazed states resem- 
bling HYSTERICAL FUGUES. Probably an in- 
stance of NEUROSYPHILIS. 



Case 74. Abel Bachmann, a man of 40 years, remains 
doubtful and perhaps belongs to the still unresolved group 
of mental cases due to syphilis that cannot be placed in any 
of the well-known categories. Bachmann had been found by 
the police, working in front of a cowbarn without the consent 
or even the knowledge of the owner. Bachmann had, in 
fact, spent the night in the cowbarn and was working with 
the idea of paying for his night's lodgings. The situation 
struck the police as so peculiar, and Bachmann was so con- 
fused and irresponsive, that he was brought to the Psycho- 
pathic Hospital. The afternoon of his admission, however, 
he entirely cleared up and was able to give a good account of 
himself. 

His story was that he had been worrying a good deal about 
a divorce suit, and the morning of his episode he had awakened 
with peculiar feelings. He walked from Boston to Cambridge, 
feeling that he was in a strange city. He recognized the 
places he passed, yet they all seemed to be changed. Upon 
reaching Harvard Square, he determined to return to Boston 
and walked and walked, failing to reach Boston. All day he 
had eaten nothing; when night fell he stole into a field and dug 
out radishes. A postman stopped and said, " Hello, Bill," 
which awakened him as by an electric shock. A barn pre- 
sented itself, in which he spent the night. In the morning, 
the barn looked different. In fact, his entire surroundings 
appeared mysterious. As he felt like working, he went to 
work in front of the barn. 

It seems that in his life there had been two other episodes 
of a similar nature; in fact, Bachmann had been in a state 
hospital for six weeks after the first episode. The first 
episode had lasted a few days only, and followed worry when 
he learned that the girl with whom he was in love was married. 



PUZZLES AND ERRORS 265 

The second attack followed the death of his mother, where- 
upon he was taken to a state hospital although the total 
duration of symptoms was only three days. Bachmann 
had had a chancre or some other form of genital disease at 
26, and had at that time been treated with mercury. 

Except for irregular and absolutely rigid pupils, reacting 
neither to light nor to accommodation, Bachmann showed 
no physical and especially no neurological disease whatever. 
Moreover, the W. R. in the blood serum was negative. 

As to diagnosis, one might consider hysteria, of which, 
however, there are no visible stigmata. It would not appear 
that brain tumor would be likely to have lasted so long as 
eight or nine years, even if we should attempt to make the 
hypothesis of tumor cover both the non-reacting pupils and 
the episodes. Bachmann was non-alcoholic, and there was 
no sign of any other form of intoxication. The spinal fluid 
showed a negative gold sol reaction, there were no cells in the 
fluid, there was no globulin; albumin was normal. How- 
ever, the W. R. was strongly positive. 

The situation, then, in this case is that we have somewhat 
peculiar psychopathic episodes, pupils rigid to light and 
accommodation, a positve W. R. in the spinal fluid, and ex- 
tremely little else to permit a diagnosis. We are ignorant 
as to the course and pathology of such cases. However, we 
cannot resist the temptation of the diagnosis of neurosyphilis, 
although further classification is not ventured. 

I. What is the significance of stiff pupil as an isolated 
symptom? Nonne finds that in the end, after years of 
observation, the Argyll- Robertson pupil turns out to 
be an advance courier of other more functionally 
serious signs and symptoms of neurosyphilis. We can 
confirm this experience and regard it as an established 
clinical proposition that the Argyll- Robertson pupil 
cannot be neglected. In this connection, refer to the 
case of alcoholic pseudoparesis (Murphy, 60), and also 
to the case of pineal tumor (Donald Falvey, 35). En- 
thusiastic reports have occasionally been made upon 
apparent restoration of the true syphilitic Argyll- 
Robertson pupil to normal light reaction. The diffi- 
culties in rendering the symptomatic diagnosis of 



266 PUZZLES AND ERRORS 

Argyll- Robertson pupil in a given case are so great, and 
the chances of complication so numerous, that we are 
inclined to attach little significance at present to these 
claims. 

It may not be amiss to mention a somewhat humorous 
incident familiar to some local neurologists. A case was 
reported by the interne for a number of months as a 
victim of a pupil stiff to light and accommodation, and 
the entirely adequate cause of this phenomenon was 
actually only discovered at autopsy by the triumphant 
medical examiner, who demonstrated that the patient 
in question was possessed of a glass eye. 



PUZZLES AND ERRORS 267 



TABETIC NEUROSYPHILIS ("tabes dorsalis ") 
versus PERNICIOUS ANEMIA with spinal 
symptoms. 



Case 75. Mrs. Brown was a woman of 56, who for the 
past eight or ten years had been complaining of trouble in 
her legs. As she described it, at times her legs were so weak 
she could hardly stand; at other times there was consider- 
able pain and numbness. She has always been considered 
"high strung"; that is, she had a very bad temper and 
lost control of herself almost entirely when she became 
excited. Her legs had been growing progressively worse, 
and for about a month prior to admission she had been un- 
able to stand or walk. She had also lost control of her 
bladder. On account of her temper, it had been almost 
impossible to nurse her; no nurse would stay with her be- 
cause of her scolding and fault-finding. Recently, she had 
been having fits of the blues. 

Her husband, who was seen before Mrs. Brown, was an 
old gentleman, over 70, who was chiefly remarkable from the 
fact that he had unequal, irregular pupils, which reacted 
neither to light nor accommodation ; there was also a speech 
defect. 

The patient herself proved to be extremely irritable, as 
had been stated, — so much so that at times it seemed 
almost impossible to do anything for her. She was very 
querulous, constantly complaining, and not satisfied with 
anything that was done. Aside from this, her mental ex- 
amination proved to be entirely negative ; that is, there were 
no psychotic symptoms. 

The systematic physical examination gave the following 
significant findings: blood pressure, 160 systolic, 90 diastolic; 
no evidences, however, of peripheral arteriosclerosis. Patient 
was unable to walk or stand, and had no control over her 
bladder. The knee-jerks and ankle- jerks absent on both 
sides ; ataxia in the leg movements ; loss of sense of localiza- 



268 PUZZLES AND ERRORS 

tion, with no tenderness over the nerve trunks; no atrophy, 
paralyses, or muscular asymmetry of the parts. The vibra- 
tory sense was maintained. Subjectively, the patient thought 
that the vibratory sense differed in the legs from that in the 
arms. Localization, touch, pain, heat, and cold responded 
to correctly. The arms showed nothing abnormal; there 
was no incoordination, dysmetria, or dysdiadochokinesis. 
Her pupils were equal, regular, and both reacted normally 
to light and accommodation. 

Diagnosis : The first consideration in the case is naturally 
tabes dorsalis, especially when one considers that the hus- 
band had signs which suggested syphilis of the nervous 
system. The rapid onset of the acute symptoms in this 
case, and the absence of the characteristic signs of pain were 
at least atypical for this diagnosis, as was the absence of 
any pupillary signs. Further, the W. R. was negative in 
the blood and spinal fluid ; there were no definite signs of 
inflammatory reaction as shown by the other spinal fluid 
tests. These findings made a diagnosis of tabes entirely im- 
probable. After tabes, the most frequent cause of the symp- 
toms above enumerated is perhaps to be found in pernicious 
anemia. Examination of the blood showed that the patient 
had 2,500,000 erythrocytes per cmm. The hemoglobin by 
Tallquist scale was 80%. The smear was practically nega- 
tive; no blasts could be seen. Although this picture is not 
a typical one for pernicious anemia, at least it is significant 
in the low number of red cells to be found, and as no causes 
for anemia were to be found, it seemed probable that we 
were dealing with a primary anemia. The diagnosis in the 
case, therefore. Is spinal sclerosis of primary anemia. The 
mental picture was not uncharacteristic of Pernicious 
Anemia. 

I. Could the diagnosis be rendered in this case without 
a lumbar puncture? In the first place, the emaciation 
is not entirely characteristic. The pupils react nor- 
mally to light. Probably such a case might well 
have been regarded as one of tabes dorsalis in former 
days, or even at the present time, if a lumbar puncture 
had not been resorted to. 



PUZZLES AND ERRORS 269 

2. Could this case possibly have been one of tabes dorsalis 

with negative spinal findings? Such cases have been 
reported frequently, but, unlike the present case, are 
apt to be of long standing and non-progressive, in 
which the active inflammation is no longer present. 
The negative findings would not be consistent with 
tabes, in which the symptoms are of short duration 
and of sudden onset. 

3. If the serum W. R. had been positive would the diag- 

nosis have been neurosyphilis? We are loath to make 
the diagnosis of spinal syphilis when the spinal fluid is 
normal. Syphilis may produce a marked anemia, 
however, and thus produce symptoms such as shown 
by Mrs. Brown. It is even possible that such is the 
explanation of this case, taking into consideration the 
suggestive findings in the husband. However, there 
is insufficient evidence to make such an hypothesis 
rock firm, and we do not more than suggest it. 



270 PUZZLES AND ERRORS 



Atypical case of CONGENITAL NEUROSYPH- 
ILIS — peculiar mental state. 



Case 76. James Seabrook, 20 years of age, obviously 
showed a number of signs of congenital syphilis. The 
physical examination disclosed an old scar and indentation in 
the right mastoid region, another on the right side of the 
neck, another on the posterior surface of the right forearm, 
and two on the outer surface of the right upper arm. The 
lesions were about the size of half a dollar. There was a 
saddle-shaped nose and a perforation of the palate and 
uvula ; there were palpable cervical and axillary glands, small 
but numerous. There was a dulness In the region of the right 
scapula, and slight dulness on both sides behind. There were 
loud whispering and piping rales and bronchial breathing 
throughout the chest, more marked on the left; there was 
much coughing, and the sputum was at times blood-stained. 
The pupils were Irregular but reacted perfectly. The left 
knee-jerk was slightly more active than the right. The W. 
R. In blood and fluid was negative; the gold sol, globulin and 
albumin tests were negative. There were, however, 56 
cells per cmm. In the fluid. 

We learned that the patient had had several spells of great 
excitement, with pounding on the door and a desire to fight 
bystanders. There were spells of headache and vertigo. 
Mentally the tests showed him to be subnormal. 

The diagnosis of Congenital Syphilis seems established ; 
possibly the pulmonary condition is syphilitic. The mental 
subnormallty as well as the abnormal traits and episodes 
are probably to be accounted for on the basis of syphilitic 
involvement of the brain. 

1. Are the headache and vertigo connected with syphilis? 

This is perhaps suggested by the pleocytosis in the 
spinal fluid. 

2. How shall we explain the negative W. R.? This patient 

had received, shortly before his entrance to the hospital, 



PUZZLES AND ERRORS 27 1 

salvarsan and mercury. Possibly the drug treatment 
has Uttle or nothing to do with the negative W. R.'s 
since they not infrequently grow weaker as congenital 
syphilitics grow older. 

What is the explanation of the spells of excitement? 
Compare the spells of excitement in a form of neuro- 
syphilis described by Kraepelin, namely: syphilitic 
paranoia, discussed in the case of Bridget Collins (59). 

Is treatment indicated considering the W. R.'s to be 
negative in blood and fluid? Despite the negative 
jW. R.'s in this case treatment is strongly indicated 
on account of the pleocytosis. This would seem to 
indicate that there is an active inflammatory process 
in the cerebrospinal axis, and it is more than probable 
that this process is syphilitic. How much real im- 
provement of the symptoms would result from anti- 
syphilitic treatment it is impossible to prophesy. 
Every case is a special problem, and this case is very 
unusual in showing a pleocytosis in the absence of 
other indications of syphilitic nervous disease, viz., 
globulin, albumin and W. R.'s. 



272 PUZZLES AND ERRORS 



CONGENITAL NEUROSYPHILIS resembling 
an undifferentiated case of FEEBLEMINDED- 
NESS— actuaUy PARETIC. 



Case 77. John Friedreich, a 7-year old boy, was brought 
to the Psychopathic Hospital by agents of a charitable society, 
who found him a neglected child and quite evidently a sub- 
normal one. 

The dominance of syphilis in the situation was clear. The 
boy's father had died but a few months before of syphilitic 
heart disease, from which he is said to have suffered for five 
years. The boy's mother (the parents were first cousins) 
had also been treated for syphilis and was excessively al- 
coholic. The first child of this union — a girl — had died 
at 6 years, of a disease diagnosticated spinal meningitis. 
The history indicates that syphilis was acquired after the 
birth of this first child; but in any event it is possible that 
the meningitic condition of which the first child had died was 
syphilitic. The second pregnancy terminated in a still- 
birth; the third issued in a girl, who died two weeks after 
birth of what was termed " inward convulsions." The 
fourth pregnancy resulted in a miscarriage ; the fifth in our 
patient, John Friedreich. The sixth pregnancy resulted in 
a girl, now 5 years of age, who is apparently normal. (Her 
W. R. was negative and she shows no stigmata of syphilis.) 

The patient, John Friedreich, at some very early age had 
a rash on his body diagnosticated as syphilis. He also had 
many seizures called fainting spells. Ever since birth he had 
been taking mercury pills. He had not learned to talk 
until his third year, and was able then to say only a few dis- 
connected words. In fact, John has never been able to talk 
in complete sentences, mumbling much that is quite unin^ 
telligible. However, he walked at 15 months in a normal 
fashion and nothing peculiar in his gait was noted until he 
was 5 years old, when he began walking on his toes, par- 
ticularly those of his left foot. Shortly thereafter, the seem- 




4 ' 




Juvenile paresis. 7 years. 



PUZZLES AND ERRORS 273 

ingly inevitable trauma appeared ; John fell out of a window 
and severely injured his left leg, whereupon the peculiarity 
of toe-walking became more pronounced and associated 
with a limp. 

The patient strikes one physically as having the develop- 
ment of a child of about five years (actual age, 7). There 
are a few lymph nodes palpable in the anterior triangles of the 
neck. The dilated and slightly unequal pupils react neither 
to light nor accommodation. There is practically complete 
deafness; loud sounds are not at all noticed. 

Withal, the child in a general way presents a somewhat 
attractive appearance, being very playful and mischievous, 
lying about on the floor and playing with whatever comes to 
hand, talking to himself or making a few indistinct remarks 
to the bystanders. He walks awkwardly, on the toes of the 
left foot. He pays little or no attention to his toilet and 
needs to be dressed and cared for in all ways. He is quick- 
tempered and at times very difficult to manage. 

There was, of course, little doubt of the diagnosis of Con- 
genital Syphilis and of Feeblemindedness. The W. R. 
was positive both in the blood and in the spinal fluid. The 
gold sol reaction of the fluid was of the " paretic " type; 
there were 44 cells per cmm. and there was a large excess of 
albumin and much globulin. 

As to prognosis, there is doubt. 

1. Is, or is not, this a case of juvenile paresis? 

2. Is it, perhaps, a relatively permanent case of feeble- 

mindedness due to congenital syphilis? On the whole, 
on account of the spinal fluid symptoms, we should be 
inclined to give the case a relatively poor prognosis, 
namely, of death in a few years. However, we may 
perchance be later surprised to learn that the patient 
has lived on, at least into early adult age. 

Note: Mercury tablets in some cases of congenital 
syphilis do not seem effective. John Friedreich was 
treated most Intensively by syphllographers from birth. 

Dr. W. E. Fernald in a personal communication stated 
that syphilitic cases of feeblemindedness are rather those 
of the Imbecile and Idiot groups than of the higher levels. 
This statement emphasizes again that the true hereditary 



274 



PUZZLES AND ERRORS 



cases of feeblemindedness are rather those of the higher 
group, whereas the cases in which special causes have 
operated in the uterus or in early life eventuate in idiocy 
and imbecility. However, such a case as that of Fried- 
reich shows that now and then a case of feebleminded- 
ness without evidence of neurological disorder and look- 
ing in almost all respects like an hereditary case may be 
at times produced by syphilis. 
How often is the central nervous system involved in 
hereditary syphilis? An interesting table bearing on 
this point is presented by Veeder.* The table concerns 
the lesions in various parts and systems of the body in 
lOO cases of late syphilis. It appears that in 47, or 
approximately one-half of Veeder' s series of 100 late 
cases, the infection developed some form of lesion of 
the nervous system. As Veeder remarks, this result 
runs counter to the common statements of pediatri- 
cians, notably of Holt. 



Bones: 

Periostitis tibia ...... 4 

Periostitis skull I 

Osteomyelitis i 

Joints: 

Acute arthritis knee . . 8 

Acute arthritis ankle. i 

Skin: 

Macular eruption .... i 

Condyloma anus 3 

Gummata 3 

Alopecia 3 

Eye: 

Interstitial keratitis . . 24 

Choroiditis i 

Ulcerations : 

Nasal 2 

Laryngeal i 

Pharyngeal i 



Central Nervous System : 

Mental deficiency 23 

Cerebrospinal syphilis 14 

Hemiplegia 6 

Epilepsy 5 

Spastic paraplegia 4 

Chorea 2 

Hydrocephalus 2 

Miscellaneous Conditions: 

Ozena 

Enlarged spleen (only symp- 
tom) 

Torticollis 

Aortitis 

Obscure abdominal pain. . . 

Obscure pain in legs 2 

Endarteritis obliterans .... 
Paroxysmal hemoglobinuria 

Raynaud's disease 

Hutchinson's teeth 4 



* Borden S. Veeder: Hereditary Syphilis in the Light of 
Recent Clinical Studies; Am. Jour, of Med. Sc, CLII, 1916. 



PUZZLES AND ERRORS 275 



Juvenile paretic neurosyphilis. Quadriplegia. 



Case 78. Gridley Ringer, 15 years of age, had the facies of 
a congenital syphilitic, including Hutchinsonian teeth, rhag- 
ades of the face, and the so-called Olympic brow. No 
secondary sexual characteristics had developed. There was 
a marked speech defect. Mentally, Ringer was a low-grade 
imbecile. He had been born at full term, and delivery had 
been normal. There had never been other pregnancies. 
He had never developed normally. 

The father admitted syphilis 23 years before, namely, 
8 years before the birth of his son, but the father had been 
treated for several years and had been declared cured. 

1. What would be expected in the spinal fluid of this case? 

Without the history, it would perhaps be impossible to 
say whether the case was one of a quiescent imbecility 
or one of juvenile paresis. The spinal fluid of the juve- 
nile paretic gives a picture identical with that in the 
adult. The spinal fluid in this case showed a positive 
W. R. (as did also the serum), a marked increase of 
albumin and globulin, 115 cells per cmm., and a " pa- 
retic " gold sol reaction. Accordingly, the diagnosis of 
General Paresis was made. 

2. What is the prognosis? The prognosis of juvenile paresis 

is currently regarded as entirely grave. There is 
probably less hope for Improvement in juvenile paresis 
than In the acquired paresis of adult life, since it seems 
to be a general principle that congenital syphilis is 
always more difhcult to cure than acquired syphilis. 

This case had seizures a few months after initial 
observation, and the seizures were followed by a tran- 
sient right hemiplegia. This right hemiplegia was 
shortly followed by a left hemiplegia, which remained 
permanently. Moreover, a few weeks later, a right 
hemiplegia again developed, leaving the patient with 
complete paralysis and aphasia. Death followed in 
six weeks. 

3. What effects were shown in the parents? Following up 

the parents was rewarded by the discovery that the 



276 PUZZLES AND ERRORS 

mother was suffering from nerve deafness, probably 
of syphilitic origin, and that the father had recently 
begun to suffer from what he considered rheumatism, 
but which on examination was shown to be tabetic 
neurosyphilis (" tabes dorsalis "). This family again 
supports the hypothesis that there is a strain of spiro- 
chetes especially prone to attack the nervous system. 
Here it would seem that the syphilis acquired by the 
father had infected the mother and been transmitted 
to the son. In all three infected by the same strain 
or strains of organisms the nervous system was in- 
volved. It is difficult, nevertheless, to explain on this 
hypothesis why in one case the disease took the form 
of tabes dorsalis, in the second, eighth nerve involve- 
ment and in the third, paresis. This question of strains 
is really more than academic because it enters deeply 
into the question of treatment, as well as that of the 
suggested increased viability of the neural strain. 



PUZZLES AND ERRORS 277 



Is there a relation between epilepsy and juvenile 
neurosyphilis? 



Case 79. John Doran fell off the rear of an ice-wagon, at 
six years of age, and shortly afterward developed fits. It 
appears that John was not unconscious at the time of his fall, 
but that he complained of headache. Although the convul- 
sions were fairly frequent at first, it appears that they later 
became rare and occurred only when the patient got into a 
temper. At the stage of exhaustion after violent excite- 
ment, John would fall. 

Physically, at 9 years a fair development and nutrition 
were evident. There was a great exaggeration of the frontal 
bosses; the nose could not be said to be typically saddle- 
backed, yet there was a suggestion of a sinking of the 
bridge. The teeth slightly suggested the Hutchinsonlan 
type, but only slightly. There was a slight roughening of the 
tibia, and there was a slight scar over either knee. The 
patient graded according to the Binet scale at 9 years, and 
he was regarded as definitely feebleminded. 

The family physician states that, according to his informa- 
tion, the father contracted syphilis when the child was between 
three and four months of age, and that the mother also was 
infected at this time. However, the child had not been 
suckled except immediately after birth, and there had been 
no evidences, according to the family physician, that John 
had acquired syphilis. 

Ordinarily, one might content himself regarding the case 
of John Doran as one of Idiopathic epilepsy with mental 
defect or deterioration. However, the frontal bosses, sug- 
gestive teeth, the flattened bridge of the nose, the roughened 
tibiae, and the old scars, though singly not of great significance, 
collectively make one suspicious. Despite the family physi- 
cian's belief that John could not have acquired syphilis from 
the parents, the infection seems entirely possible despite the 
fact that no symptoms developed early thereafter. 



278 PUZZLES AND ERRORS 

The W. R. in this case proved positive in both blood 
serum and spinal fluid. 

1. What is the relation of trauma to this case of Juvenile 

Neurosyphilis? Probably none. 

2. What would be the effect of treatment? For a number of 

years John Doran was lost sight of. He was, how- 
ever, treated, according to our information, with intra- 
spinous injections of salvarsanized serum, whereupon 
his convulsions shortly ceased. He has been recently 
examined mentally once more, and still grades as feeble- 
minded. He still has violent outbreaks of temper. 

3. Is such a case as Doran typical? Shanahan has investi- 

gated conditions at Craig Colony. There were 22 
out of 886 epileptics (at Craig Colony) or 2|%, who 
showed a positive W. R. Nine of these cases were 
regarded by Shanahan as cases of epilepsy actually 
caused by syphilis. Viet had found 7%, and Bratz and 
Liith 5% of constitutional epileptics to be syphilitic, 
but the data of these German authors were obtained 
before the era of Wassermann tests. 



PUZZLES AND ERRORS 279 



Adrenal tuberculosis complicating juvenile paretic 
neurosyphilis (*' juvenile paresis "). Autopsy. 



Case 80. When James Arnold appeared at the Dan vers 
Hospital in his 22d year, he looked as if he were but 12 or 14 
years of age. He was excessively fat but of fair muscular 
development. The left eye diverged outward, and the left 
pupil was smaller than the right. An odd feature was a high 
degree of pigmentation of the skin of the genitalia and the 
groins (the axilla, the mammillary areas, and the oral mucosae 
were free from pigmentation). Physically speaking, the 
patient was practically normal. Neurologically, however, 
there was much of interest, in the light of which the clinical 
history was of value. 

It seems that after an apparently normal early childhood, 
the boy had begun, at the age of 11, to experience difhculty 
in carrying out every-day school tasks; and after this his 
mental capacity had slowly but progressively deteriorated. 
The deterioration was not merely Intellectual, but the boy 
became dishonest and untrustworthy and developed a num^ber 
of untidy and uncleanly habits, behaving at the age of 16, as 
the parents stated, like a child of six. 

In his seventeenth year, the boy had been taken with a severe 
attack of what was regarded as an " attack of Indigestion." 
This attack ushered In a gradually developing muscular 
weakness, especially Involving the limbs. By the age of 21 
he had become irritable and the paresis was so extreme that 
the patient was unable to get In or out of a carriage. 

This generalized muscular weakness was plain upon ad- 
mission to the hospital though there seemed to be no actual 
paralysis. The patient was unable to walk In a straight line 
and Romberg's position could not be maintained. Marked 
tremor was present In the hands and lips. There was bi- 
lateral impairment of vision and nystagmus. Reflexes and 
sensations normal. Speech was markedly affected, all sylla- 
bles being very much slurred. School knowledge and memory 



280 PUZZLES AND ERRORS 

for both recent and remote events very poor. The patient's 
habits were very untidy. He was very emotional, easily 
made to laugh or cry; and in behavior, extremely childish. 

Two months after his admission to the hospital, the weak- 
ness suddenly became extreme. He was constantly nau- 
seated, refusing food. The face and hands were cyanosed and 
the heart's action rapid, weak, and irregular. This attack 
lasted for a week and was followed by a period of improve- 
ment, during which, however, he still remained very weak and 
apathetic. 

One month later he again became so feeble that he was 
unable to raise himself in bed. He complained persistently of 
feeling very " sick." His temperature was elevated and there 
occurred the same train of circulatory symptoms observed 
previously, viz., rapid and tumultuous action of the heart, 
with cyanosis of face and extremities. He soon became 
unconscious, remaining so until his death, which occurred 
on the seventh day of the acute attack. 

This case was under observation before the days of the 
W. R., yet clinically the case had been diagnosticated Juve- 
nile Paresis. There was no history of the acquisition of 
syphilis or any likelihood of its acquisition. Considered clini- 
cally, many of the classical features described by Addison 
were present, viz., marked asthenia and apathy; severe and 
frequent gastro-intestinal symptoms (the disease probably 
commencing with the attack of so-called " acute indigestion " 
six years prior to patient's death) ; attacks of extreme cardiac 
weakness with the characteristic small, low-pressure pulse. 
On the other hand, pigmentation of the skin (usually the 
most striking clinical feature) was limited to the external 
genitalia, these being colored a deep brown. 

The most striking feature found at autopsy was a bilateral 
adrenal tuberculosis (caseation, giant cells, lymphocytosis, tu- 
bercle bacilli). The thymus gland was persistent (7X5X.5 
cm.), whereas the thyroid gland was smaller than usual. 
The brain showed macroscopic and microscopic features 
entirely consistent with the diagnosis of general paresis, 
including lymphocytosis, plasmocytosis, irregular degrees of 



PUZZLES AND ERRORS 28 1 

nerve-cell destruction, and gliosis, with an especially charac- 
teristic microscopic picture in the frontal regions. 

It may be of note to consider the degree of change under- 
gone by a brain in 1 1 years or more of deterioration, and the 
following description of the head findings is therefore 
included : 

Head: Hair abundant, dark. Scalp normal. Cal- 
varium, weight 435 gm., transparent in bregmatic region 
only, elsewhere thick and dense. The average thick- 
ness of the vertical plate of the frontal bone is 7 mm. 
The frontal bone shows a moderate thickening and 
hardening of the inner table with obliteration of diploe. 
Dura mater moderately adherent to the bregmatic 
region of calvarium. Arachnoidal villi moderately 
developed. Sinuses not remarkable. Pia mater shows 
a moderate focal thickening with opacity, especially 
along sulci. Vessels well injected. Brain: Weight, 
1200 gm. The brain shows marked focal variations 
in sulcation and consistence. Spread on a board, the 
right hemisphere is obviously somewhat bigger than 
the left. There is a difference of only 0.5 to 0.75 cm. 
on measurement of the greatest circumference of the 
cerebrum, taken from the median line superiorly to the 
median line inferiorly, but the right hemisphere is 
throughout slightly more convex than the left. Both 
postcentral gyri are much narrowed in their superior 
portions, and the sulci posterior thereto are deeper 
than the other sulci of the hemispheres. The sulci 
of the orbital surfaces are asymmetrical and, on the 
left side, show a tendency to microgyria. The cerebral 
hemispheres as a whole show a remarkable tendency to 
slight protrusion of the border gyri ; especially those of 
the two poles, of the free edges along the great fissure, 
and most strikingly the gyri at the boundary line between 
the inferior and lateral surfaces. This marginal prom- 
inence is slight but obvious and is emphasized by a 
slightly paler color in some regions. The cerebrum 
shows a general induration which is greatest in the frontal 
tips and along the inferior borders of the lateral surfaces 
of the hemispheres, especially right. The orbital sur- 
faces are firm, especially anteriorly and externally (pre- 
frontal) ; the tips of the temporal lobes are firm, and 
the superior temporal gyri are firmer than adjacent 
gyri. The postcentral gyri are indurated more than 



282 PUZZLES AND ERRORS 

the other gyri of the superior surface. The hippo- 
campal gyri are Hkewise firmer than adjacent gyri. 

Cerebellum and pons: Weight, 145 gm. The in- 
equaUty of the two hemispheres is more marked than in 
the case of the cerebrum. 

Greatest lateral diameter; left, 4.5 cm., right, 5.5 cm. 

Anteroposterior diameter adjacent to notch: Left, 
5.8 cm., right, 5.5 cm. ^ 

There is no appreciable difference in depth. The 
diminution in volume appears to be chiefly at the expense 
of the right clivus. The inferior surface is firmer than 
the superior. The laminae adjacent to the horizontal 
fissure are firmer than the remainder of the cerebellum. 
The pons is small. 

There was also a lateral curvature of the spinal column, 
as well as characteristic adhesions between spinal dura and 
pia mater which are always suggestive of syphilis. For the 
rest, there were few findings of note: some adhesions of the 
left pleura, hypostatic congestion of the lungs, tracheitis, and 
chronic gastritis. There were four lobes of the right lung 
but it is doubtful whether this should be regarded as a stigma. 

1. Can we separate the symptoms of Addison's disease from 

those of paresis in this case? The extreme cardiac 
weakness with a characteristic, small low-pressure 
pulse is in point. The asthenia and apathy are consis- 
tent enough with Addison's disease as well as with paresis 
itself. It would also be possible to ascribe the gastro- 
intestinal symptoms to either disease. 

2. Of what significance is the persistent thymus? Persistent 

thymus has been observed in a few cases of Addison's 
disease, but that it plays any part in the symptoma- 
tology thereof is a matter of doubt. 

3. How can the obesity be explained? It is of course of 

note that the thyroid gland was small, but micro- 
scopically there were no peculiar features in this gland. 

4. Was the adrenal tuberculosis actually primary? Minute 

search failed to reveal evidences of tuberculosis else- 
where unless we regard the few adhesions binding the 
lower half of the lung to the chest wall as indicative 
of an old tuberculosis. In particular, the mesenteric 
lymph nodes were normal. 



PUZZLES AND ERRORS 283 



Neurosyphilis? Secondary stage of syphilis. 



Case 81. Florence Fitzgerald, a woman 25 years of age, 
applied at the police station to be taken care of. She said 
she had been a prostitute for the last few months, was now ill, 
and wanted to reform. She appeared physically ill and was 
sent to the Psychopathic Hospital, where she remained at 
first almost mute, making answers chiefly by nodding the 
head. She gave the impression of daze or stupor, and in 
fact her condition was at first regarded as catatonic. This 
reaction, after a few days, changed and Florence became 
quite normal, giving a full account of her condition. 

It seems that four months before going to the police station, 
she developed a chancre, which was locally treated. A 
careful physical examination showed a fine red macular 
eruption which was without much question a syphilitic 
roseola. The spinal fluid yielded a positive W. R. although 
other tests of the fluid were negative. Curiously enough, no 
physical sign of involvement of the nervous system could be 
discovered. We were inclined to regard the mental symptoms 
as partly due to the syphilitic intoxication, and partly due to 
a psychic reaction of the nature of defense. As for the posi- 
tive W. R. in the spinal fluid, in early secondaries various 
observers differ as to the frequency both of the W. R. and 
of other changes, percentages being given that range from 
25 to 90%. See case Caperson (45). It is of note that 
clinically there were symptoms referable to a syphilitic in- 
volvement of the nervous system; namely, marked headache 
and malaise. The headaches of the secondary period are fre- 
quently the result of meningeal involvement. 



284 PUZZLES AND ERRORS 



TABOPARETIC NEUROSYPHILIS (" tabo- 
paresis ") ; death from TYPHOID MENINGITIS. 
Autopsy. 



Case 82. Frederick Estabrook was a salesman, who, be it 
noted, had never had typhoid fever or any disease remotely 
resembling typhoid fever. He had acquired syphilis at 19; 
had married at 22; was the father of two healthy children 
(no miscarriages) ; had had a certain disturbance of bladder 
and rectum, but remained a successful salesman to the age of 
28, when advancing tabes confined him to bed for a time. 
At 30, mental signs of Paretic Neurosyphilis developed, 
and death followed at 32, after an acute illness of a week. 

The details of the history after the first symptoms at 28 
are as follows : 

At twenty-eight patient lost control of limbs and was con- 
fined to the house about two months, under medical care. 
Three months later he had regained partial control of his 
limbs but had lost all control of his sphincters. After another 
month he had returned to work, but did not work steadily 
and seemed to have lost ambition. In the summer of 1905, 
his mind became obviously altered. He grew indolent and 
extravagant and given to buying expensive and useless ar- 
ticles. Loss of interest in things followed, together with loss 
of memory for recent events, lack of insight into illness, delu- 
sions of persecution by wife, irascibility followed quickly by 
crying. Before admission to hospital, he was euphoric, drawl- 
ing and tremulous in speech, sprawling in penmanship, alter- 
nately depressed and exalted in manner. Knee-jerks were 
absent, gait ataxic, pupils stiff to light. 

The family history was negative with respect to insanity. 
All the family were reported as nervous. A brother died of 
peritonitis at twenty-eight, a sister of pneumonia under twenty. 
Another brother and sister are living. Father and mother died 
of heart trouble at about sixty-seven and sixty respectively. 

The patient was at high school one year and was a fair 



PUZZLES AND ERRORS 285 

student. Considerable tobacco was used, and some alcohol. 
Intoxication denied. There was no history of typhoid fever 
or other acute disease. 

The patient on admission was sallow, poorly nourished, and 
flat-chested, with a slight lateral curvature. There was 
slight dulness over right apex in front and in right upper 
back. Voice sounds were increased over right apex in front 
and over whole right back. The right chest showed bronchial 
respiration throughout. The respiration in front of right chest 
was of an interrupted character. The liver seemed moder- 
ately enlarged. The urine showed a very faint trace of 
albumin. There were a few small nodes in right groin and a 
scar on dorsum of penis. 

Neurological Examination. Slight swaying In Romberg 
position. Slight tremor of protruded tongue and extended 
fingers. Pupils irregular, left slightly larger than right. Left 
pupil reacted to light consensually, but not directly. Right 
pupil reacted very slightly to direct light, not consensually. 
Knee-jerks and Achilles jerks absent. Ankle clonus absent, 
abdominal and cremasteric reflexes brisk. Sharp and dull 
points were recognized in the legs with numerous mistakes. 
Vocal and facial tremor. Speech slow and drawling. Test 
phrases repeated well if care was taken. Consciousness clear. 
Orientation perfect. Calculating ability preserved. Many 
words omitted in writing. Penmanship clear but shaky. 

Hallucinations absent. Memory of recent events poor. 
Associations of a logical or defining type. Patient denied 
various statements in commitment papers and had little or no 
insight Into the mental side of his disease — slight euphoria. 

After a month's observation the patient was removed to a 
quiet ward and set to work a few days in the scullery. One 
night he began to yell as if assaulted and said later that he 
had an Idea that he was going to die. Before three months 
had passed he had become untidy, disorderly, and imperfectly 
oriented. 

The general degeneration continued rapidly. One week 
before death the temperature rose to 103 degrees F., and the 
patient succumbed to what seemed clinically like a broncho- 
pneumonia. Unconsciousness two days before death. 



286 PUZZLES AND ERRORS 

Note with respect to history of typhoid. — Inquiries of 
his physicians, wife, employer, and brother tend to show 
conclusively that the patient never had a disease even re- 
motely resembling typhoid fever. 

The autopsy findings were as follows: 

Acute conditions: 

Hypostatic pneumonia, with early serofibrinous pleuritis 
and without lymph node swelling; enlargement of mesenteric 
lymph nodes; acute cerebrospinal leptomeningitis; multiple 
small hemorrhages of spleen. 

Other findings: 

Scar of penis; sclerosis of aortic arch (Heller's type?) and 
slight coronary arteriosclerosis; calvarium thin and dense; 
dura mater thickened and adherent to calvarium; calcified 
arachnoidal villi; chronic cerebral and cerebellar leptomen- 
ingitis; atrophy of frontal lobes; granular ependymitis; scle- 
rosis of posterior columns of spinal cord ; emaciation ; unequal 
pupils; slight parietal fibrous endocarditis, slight mitral scle- 
rosis; gastrointestinal atrophy; chronic cystitis; chronic ab- 
scess of prostate. 

The description of the head findings is as follows: 
Skin exceedingly loose, and the whole skull cap 
thinned. The diploe are absent. Adhesion with dura 
easily separated. The dura somewhat thickened, but 
not distended. Along the longitudinal sinus extensive 
calcareous granulations adhere to It. The longitudinal 
sinus does not contain blood, and the Inner surface 
is normal In color. The pla is extensively thickened 
and opaque and a general subplal exudate exists which 
is more marked over the vertex where It lifts the pla 
from the brain surface to the extent of three centi- 
meters in Rolandic, superior frontal, Intraparletal, 
and mesial precentral sulci on each side. The arteries 
at base are free from atheroma. The temporal lobes 
are much bound down by adhesions, as Is the cerebellum. 
Post-mortem softening is evident. The hemispheres 
show no asymmetry, but the frontal convolutions are 
markedly atrophic. The corpus callosum is united to 
the cortex by old adhesions and has to be dissected 
away from it. Lateral ventricles contain some slight 
amount of cloudy fluid, and the pla along the vessels 



PUZZLES AND ERRORS 287 

is Opaque. Some granulations in ependyma. Brain 
weight, 1305 grams. Pons and cerebellum, 195 grams. 

Cord. — Dura much thickened, and the pia corre- 
sponds to its appearance in brain with a like exudate. 
Cross-sections of cord show sclerosis of posterior 
columns. 

Bacterlologically the typhoid bacillus was cultivated 
from the meninges and from the swollen mesenteric lymph 
nodes. The blood was negative; the intestines were 
negative so far as lesions were concerned. 

The microscopic examination confirmed the clinical diag- 
nosis of General Paresis and of Tabes, since there was 
not only an extensive chronic encephalitis, with the usual 
lymphocytic and plasma-cell deposit and irregular gliosis, 
but also a well-marked posterior column sclerosis, not un- 
usual save in its extreme degree. 

It might be surmised that some difficulty would arise in 
distinguishing the effects of paretic meningoencephalitis from 
those of the more recent typhoidal process. The well-known 
tendency of typhoidal processes to escape polynuclear ex- 
udation, at least until frank necrosis has set in, gave rise to 
the idea that the two mononuclear pictures — that of general 
paresis and that of typhoidal processes — might be confusing. 

The picture presented by the meninges was scarcely what 
might be expected. Although numerous mononuclear phag- 
ocytic cells are everywhere found, yet the predominant 
picture is that of a polynuclear exudation. 

The polynuclear leucocytes occur in greatest numbers in 
the tissue spaces, especially in the meshes of the lumbar arach- 
noid and in the spaces of the frontal and paracentral pia 
mater. In the lumbar region of the spinal arachnoid wide 
fields occur in which the cells are almost one hundred per 
cent polynuclear leucocytes. In places phagocytic cells oc- 
cur, and in a few fields, even in the open tissue spaces, the 
number of phagocytic cells may arise to fifty per cent. 
Edema is a considerable feature in the meninges. Fibrin is 
found chiefly in the cerebral meninges and appears in numer- 
ous delicate strands in the tissue spaces. 



Moloch, horrid king, besmeared with blood 

Of human sacrifice, and parents' tears; 

Though, for the noise of drums and timbrels loud, 

Their children's cries unheard that passed through fire 

To his grim idol. 

Paradise Lost, Book I, lines 392-396 



IV. MEDICOLEGAL AND SOCIAL. 



Neurosyphilis in a public character: eloquence, 
reformatory efforts, notoriety. 



Case 83. Major Isaac Thompson, M.D., was a character. 
He had been regarded as eccentric for many years prior to 
his death at 63. In fact, it seems that there had been more 
or less definite symptoms and signs about his fortieth year. 
The doctor himself had a ready explanation for his Argyll- 
Robertson pupils; he explained that he had had a peculiarly 
heavy smallpox at about the age of 27 (which would be 
about 1872). 

The doctor had a good secondary education, he had gone 
through the Civil War as a hospital steward, went into busi- 
ness after the war, married, and then went to the medical 
school, graduating at the age of 34. He continued in prac- 
tice for a dozen years, and then gave it up. For years he 
had been especially interested in certain literary lines and 
he had published any number of pamphlets, all of a some- 
what striking description, often with a political color and 
intended to stir up reform measures. The doctor never bore 
a very good reputation, and years later it was recalled that 
certain books disappeared from libraries and their loss was 
almost certainly traced to Dr. Thompson. In general, how- 
ever, he was considered to be a rather worthy local figure. 

It is possible that a fall on the ice in his 6ist year actually 
started the fatal process, since after that time the patient 
had difficulty in walking, and a few months later developed 
periods of excitement with peremptory insistence on obedi- 
ence to his wishes. Whereas formerly the doctor had 
finished up one literary piece of work after another, he now 
began to do very scattering work. He appeared in public 

289 



290 MEDICOLEGAL AND SOCIAL 

to denounce certain financial schemes with great force and 
unusual eloquence. His eloquence was greatly compli- 
mented, and these compliments induced the doctor to a 
remarkable crusade against a certain corporation; there was 
so much truth mixed with the fiction of his eloquence that 
he obtained a considerable following in his campaign. He 
wanted to start a bureau of information for the instruction 
of the public on these matters, and he planned to put up a 
building adjoining his own home for the accommodation of 
the various clerks and writers in this bureau. However, 
before the building had been actually started, an outbreak 
occurred. 

One morning the doctor was very excitable and noisy over 
the telephone, ordering typewriters and giving directions to 
mechanics. He repaired to Boston in connection with cer- 
tain resources that he supposed (and gave others reason to 
believe) had been supplied by the Government and by a 
large newspaper. One evening he returned very late. It 
appeared that he had had a fracas at a hotel and had knocked 
down one or two colored porters, acting as though drunk. 
Upon being put to bed, the doctor talked incessantly of 
religious matters, proposing to undertake a Sunday School 
class. His interlocutor did not exhibit a particular interest 
in this scheme, whereupon Dr. Thompson threatened him 
with violence. Police and doctors were called in and a con- 
stant stream of conversation lasted for hours. The patient 
was finally brought to Danvers Hospital upon representa- 
tion by physicians, to whom he told that his luck had 
turned, that he was about to be made senator from the 
district, and that he and Roosevelt were going to break up 
the trusts, and that, as a matter of fact, he was a relative 
of Mr. Roosevelt. 

Upon admission, the patient was a well preserved and 
well groomed man with gray hair and beard. He was some- 
what pallid but his teeth were well preserved and well cared 
for, and there was little or no physical change except a slight 
hypertension. He claimed that he had suft'ered from kidney 
disease for some years, and there was in fact a trace of 
albumin in the urine. 



MEDICOLEGAL AND SOCIAL 29 1 

Neurologically, the plantar and Achilles reactions could 
not be obtained, but there were no other reflex disorders 
except the bilateral Argyll-Robertson pupil. The doctor's 
explanation for these stiff pupils, which he described as 
existing for many years, was frank and circumstantial, so 
that the unlikelihood of Argyll-Robertson pupils due to small- 
pox was rather frowned upon by him. Without entering upon 
a detailed description of the clinical symptoms and course 
of the disease which led to death a little over a year after 
admission, it may be said that the differential diagnosis lay 
between the expansive form of general paresis and a maniacal 
condition, presumably the maniacal phase of manic-depres- 
sive psychosis. From the data of a special staff meeting 
held upon the case, we learn that the diagnosis of manic- 
depressive psychosis was entertained more strongly than that 
of general paresis. Thus, for general paresis alone was the 
somewhat gradual onset with increasing excitement, accom- 
panied by expansive delusions concerning unlimited finance, 
personal over-importance, and Argyll-Robertson pupils. 
Dismissing the Argyll-Robertson pupils from consideration, 
the diagnosticians were led to see in the constant motor 
activity displayed in conveying an enormous number of 
thoughts on paper, inconsistent talking with digressions, a 
manic-depressive psychosis. There was no amnesia and no 
other sign of mental deterioration. There was a certain 
improvement early in the hospital stay of the patient. 
Consciousness was clear and orientation perfect. The de- 
lusions themselves, though extravagant, were not inconsis- 
tent or fantastic. The hallucinatory disorder was hardly 
characteristic either of manic-depressive psychosis or of 
paresis. 

The patient might be described as " interesting." A 
good preliminary training with years of travel and variety 
of occupation, furnished him with a fund of knowledge. 
An excellent memory, prompt replies and repartee, endless 
digressions with voluntary return to the original topic, 
caused him to be an amusing and even instructive inter- 
locutor. However, his commitment and confinement in the 
institution seemed always entirely wrong, and he expressed 



292 MEDICOLEGAL AND SOCIAL 

mixed feelings about the family, now being bitter against 
them, and again condoning their mistakes. The patient's 
conduct was good and he was tidy in habits, and tried as 
far as possible to conform to the requirements of the hos- 
pital. The doctor showed a marked antipathy toward a 
certain male attendant, who had removed articles from his 
clothing upon admission and had reclaimed a book on rules 
and regulations. The doctor prepared a list of 327 differ- 
ent acts of abuse, lack of care, and insubordination which 
he said he had observed in the hospital. 

In the last weeks of the patient's illness, his ideas became 
more expansive and extravagant, dealing with a grapevine 
system of wireless communication and delusions of un- 
limited wealth. He would at times keep his room flooded 
with urine and water for the purpose of keeping down the 
plague which he said was infecting the hospital. Later he 
mixed food with urine and other ingredients, claiming that 
he was constructing an elixir of life. 

The autopsy showed few changes of the calvarium or of 
the dura mater, nor was the pia mater more than slightly 
thickened and milky over the frontal poles, along the longi- 
tudinal fissure and over the sulci. There were fairly firm 
adhesions of the pia mater to the dura mater along the 
longitudinal fissure and over the frontal poles and at the 
temporal tips. The hemispheres were firmly interadherent, 
and the cerebello-pontine tissues were covered with a firm 
leptomeningitis. The floors of the ventricles were smooth 
and the basal vessels showed little beyond a few spots of 
sclerosis. There was a generalized increase of consistence. 
The frontal gyri were rather prominent with wide sulci, but 
upon section no very marked atrophy of the gray matter 
could be shown. The rest of the brain failed to show any 
flaring of sulci or any special evidence of cortical atrophy. 
The brain weighed 1250 grams; a possible diminution of 100 
grams, considering the patient's body length. However, it 
must be remembered that he was at this time 63 years 
of age. 

Microscopically, the diagnosis of General Paresis was 
confirmed on the basis of plasmocytosis, lymphocytosis, gliotic 



MEDICOLEGAL AND SOCIAL 293 

changes and nerve-cell destruction. There was an unusual 
variation in the degree of the destructive process, which 
picked out, for example, certain regions of the right side 
for maximal lesion (cornu ammonis, gyrus rectus, and su- 
perior frontal gyrus). 

If the patient's own estimate of 35 years' duration for 
his Argyll- Robertson pupils can be trusted (and in general 
his memory was extremely good), we may well conceive an 
unusual duration for the process in his case. There was, 
however, in the body at large no very marked degree of 
changes. There was a slight old tuberculosis. There was 
a slight interstitial nephritis, with cardiac hypertrophy and 
fibrous myocarditis. There was also a sclerosis of the mitral 
and aortic valves; there were chronic changes in the spleen, 
liver, and bladder; there was generalized arteriosclerosis of 
mild degree; there were two round gastric ulcers near the 
pylorus. The liver weighed but 800 grams, and its left 
lobe was somewhat rough. 

This case is placed among the medicolegal and social 
cases because the phenomena that ushered In his last illness 
were mistaken by the local public for meritorious social 
reform measures. They were regarded as not markedly 
different from the variety of steps taken by the very active 
doctor in previous years; indeed the public eloquence that 
he displayed a year before his death was quite in line with 
previous habits, despite the suspicious over-brilliance of 
language. It is an important question, how far the eccen- 
tricity and literary overactivity of the latter half of the 
doctor's total life can be explained on the basis of a mild 
syphilitic irritation of the nervous system. In this con- 
nection we are tempted to recall the suggestions of Moebius 
concerning a portion of the literary products of Nietzsche. 
Our doctor was by no means so brilliant an exemplar of 
syphilitic literature as was Nietzsche, if we grant the hy- 
pothesis of Moebius to cover our doctor's case as well as 
that of Nietzsche. In the future, important studies of 
character change under the influence of syphilis will doubt- 
less be made. With modern diagnostic methods, of course, 
the diagnosis would have been rendered almost at once in 



294 MEDICOLEGAL AND SOCIAL 

the case of Major Isaac Thompson, M.D., and much of his 
past Hfe would have been brought under special review in 
connection with the syphilis which doubtless the blood 
serum or at any rate the cerebrospinal fluid would have 
shown. 

This case illustrates but one of the many social compli- 
cations arising as the result of paresis. When one recalls 
that the onset is often insidious and not correctly under- 
stood for a period of time, it is readily seen that many unfor- 
tunate acts may be committed by a patient. As hypersexual 
desire is not an infrequent early symptom and as judgment is 
early disturbed, loose morals may ruin the patient's reputation. 
The poor judgment and expansive delusions often lead to 
foolish business deals wherein the patient's family is left 
destitute. At other times the onset is sudden and then the 
danger of false commands or acts by a person in a respon- 
sible position, as a steamship captain, an engineer or chauffeur, 
may lead to loss of life and property. 



MEDICOLEGAL AND SOCIAL 295 



Sudden grandiosity: debts. PARETIC NEURO- 
SYPHILIS (*' general paresis") : Question of liability. 



Case 84. Lester Smith was a salesman, 31 years of age, 
who, while on a business trip, accompanied by his wife, 
suddenly developed grandiose ideas. He originated a scheme 
of cornering the phonograph market. His prospects seemed 
so certain to him, that he hired an expensive suite of rooms 
in a hotel at something over $35 a day. As at the first 
presentation of his bill it was found that he had no money 
to meet these charges, he was taken into custody and at 
once transferred to a hospital for the insane, where it was 
discovered that he was suffering from General Paresis. 

I. What is the patient's responsibility for these debts? 
Legally the patient or his estate is responsible for 
debts accruing from services rendered or goods re- 
ceived. As he is adjudged non compos mentis con- 
tracts entered into would not hold, and he would not 
be considered liable for criminal acts. 

Note: This case shows how dangerous paresis may be 
not only to the life and usefulness of a patient, but further 
how it may ruin a family financially. Mr. Smith's little 
escapade used up all the money that he had been able to 
save in his life and when he was taken to a hospital his wife 
was left destitute. 



296 MEDICOLEGAL AND SOCIAL 



Suicidal attempt (?) by a neurosyphilitic. 



Case 85. At first Mrs. Annie Monks, a widowed seam- 
stress, 50 years of age, did not particularly suggest syphilis. 
Mrs. Monks was sent to us from a general hospital. She 
had been found unconscious in her room, with gas turned 
on, and a diagnosis of gas poisoning was made. Mrs. Monks 
remained unconscious for 24 hours, and her apparent sui- 
cidal attempt seemed to warrant her being sent to the 
Psychopathic Hospital. Mrs. M., however, scoffed at the 
idea of any attempt at suicide, and claimed to have had no 
recollection of any such affair. On the contrary, she had 
gone to mass the morning of the day on which she was 
taken to the hospital, remembered well enough returning 
to her room but nothing of what followed until she woke up. 

Mrs. Monks was not cooperative and would reveal few 
facts about her history. For years, she had had edema of 
the feet and palpitation of the heart (the heart was some- 
what enlarged, with a double murmur in the aortic area, 
systolic louder, and a blood pressure of 160 systolic and 
85 diastolic; clubbed fingers; palpable liver). She had 
been treated in the out-patient department of a general 
hospital for a number of months. We could obtain no 
evidence of mental impairment, particularly none of memory. 

Aside from the heart lesions above indicated, the patient 
was fairly well nourished, with a slight enlargement of 
superficial glands, and was otherwise normal. 

Neurologically, the slightly irregular pupils reacted poorly 
to light; the right knee-jerk could not be obtained, whereas 
the left knee-jerk was very active. Systematic examination 
revealed no other disorder except that the abdominal re- 
flexes could not be obtained. 

Here we have, in a cardiac patient, apossibly or probably 
accidental gas poisoning, and little to go upon for a pro- 
founder diagnosis than the sluggish irregular pupils and 
unilateral absence of knee-jerk. 



MEDICOLEGAL AND SOCIAL 297 

The routine serum W. R. came through as positive. Fol- 
lowing custom, we examined the spinal fluid, finding the 
W. R. here again to be moderately positive (strongly posi- 
tive to I cc, moderately to 0.7 cc, and negative to 0.5, 0.3, 
and 0.1 cc.)- The gold sol index was 1221000000, 
which must be interpreted as syphilitic. There were 16 
cells to the cmm., the albumin was i + , and the globulin stood 
at 2+. 

Here, then, we seem to have evidence of an inflammatory 
process of the central nervous system, and it is natural 
forthwith to be sceptical as to the accidental nature of the 
gas poisoning. Perhaps there was an attempt at suicide 
based upon a passing impulse, or perhaps there was a period 
of confusion in which the cock was not turned off. 

In any event, we feel justified in making the diagnosis of 
cerebrospinal syphilis on the basis of the neurological and 
laboratory findings. On the whole, we are inclined to 
make a diagnosis of Vascular Neurosyphilis with a mod- 
erate involvement of the Meninges. 

I. What Is the outcome In such cases as that of Annie 
Monks? The case somewhat resembled that of Martha 
Bartlett, who still survives. The case of Annie Monks 
illustrates another outcome. A few days after her 
admission, she became unconscious once more, and 
upon recovery remained very much confused and 
aphasic, moaning, and unable to handle herself well, 
although without definite paralysis. Three weeks 
later the patient died, although In the meantime 
strenuous antisyphilltic therapy was practised. Death 
was sudden. We thought death due to cerebral 
embolism. 



29S MEDICOLEGAL AND SOCIAL 



Early delinquency and neurosyphilis in a juvenile. 



Case 86. Frank Johnson was 21 years of age when he 
was taken up by the police for threatening his sister with 
a revolver. The police thought he deserved an examina- 
tion at the Psychopathic Hospital. The patient protested 
that he had threatened his sister only to frighten her be- 
cause, he said, she nagged him and made him nervous. In 
fact, they had always had trouble as she had 'always nagged 
him and they had always fought together. Moreover, their 
mother always took the sister's part. They had been troub- 
ling him for days, and at last Frank could stand it no longer. 
His sister had complained of the way he treated her dog. 
Moreover, Frank said he had not been feeling well; there 
had been some trouble with his stomach; and after one of 
the nagging attacks, he had taken out an old empty pistol 
to scare his mother and sister. 

In these cases, it is good practice to consult the sister also. 
She said that Frank had always been very difHcult to man- 
age, unwilling to work, preferring to loaf about, spending 
every obtainable cent; he was once in a reformatory for 
several years, but not reformed thereby; recently given to 
drinking; at times acting somewhat peculiarly (sitting at 
the window with his hat on, refusing to move). 

Further mental examination of Frank showed that he was 
properly oriented and in possession of a good memory, al- 
though he was quite obviously a liar. He lay about in bed at 
the hospital, saying that he was too weak to be up. He was 
a bit dull, at times not readily grasping ordinary questions. 

Physically, Johnson was rather thin; the teeth were some- 
what peg-shaped although far from typically Hutchlnsonlan. 
The pupils were unequal and Irregular, and failed to react 
to light or even to accommodation when tested. The deep 
reflexes of arms and legs could not be obtained, though the 
superficial reflexes were present. For the rest systematic 
examination proved negative. Serum W. R. negative. 



MEDICOLEGAL AND SOCIAL 299 

The first thought in such a case would be that the crimi- 
nological diagnosis of delinquency would be sufficient. How- 
ever, the pupillary disorder and the areflexia are suggestive 
despite the negative serum W. R. Resort was naturally had 
to lumbar puncture, whereupon a positive W. R. was found, 
a characteristically " paretic " gold sol reaction, globulin, ex- 
cess albumin, and 134 cells per cmm. In short, it would ap- 
pear that we must consider a diagnosis of Juvenile Paresis, 
and, in point of fact, the patient deteriorated rapidly from 
this time, becoming demented at the end of a few months. 

1. How far are the early difficulties of management (lead- 

ing to a reformatory) due to syphilis? We should not 
dogmatically say that there is a relation between the 
early delinquency and syphilis. Still, it is not unusual 
to find emotional disorder and instability as well as 
delinquency in congenital syphilitics. 

2. What suggestion, if any, should be made to the patient's 

intelligent and seemingly normal sister, two years 
older? We prevailed upon Miss Johnson to submit 
to the W. R. of the serum, which was found, as in the 
case of Frank, to be negative. Frank's sister should 
undoubtedly submit to a lumbar puncture; but in 
the present phase of mental hygiene, she would be 
difficult to persuade. 

3. How is it possible to find such a marked evidence of 

congenital syphilis in a younger sibling with no evi- 
dence of syphilis in the elder? In the first place, there 
may be a history of entrance of syphilis into the lives 
of the parents between the pregnancies. However, in 
other instances, there is no evidence of such inter- 
current syphilis, and contrary to the prevailing opinion 
it is not so infrequent to find congenital syphilis in 
the younger brother or sister of a normal person. 

4. What can be said of treatment in such cases? In the 

first place it is clear that delinquent cases should be 
tested far earlier for the possibility of syphilis. Had 
this case been examined by a neurologist or alienist 
many years earlier, it is probable that the same pupil- 
lary signs and the peg-shaped teeth would have been 
found, and that the hypothesis of syphilis might have 
been raised. There is no good evidence as yet that 
these cases can be markedly benefited by treatment. 



iir 



300 MEDICOLEGAL AND SOCLA.L 



Neurosyphilis in a " defective delinquent." 



Case 87. Vivian Walker, 22 years of age, was arrested 
on [the streets of Boston for drunkenness. .Upon arrival at 
the jail, she developed a series of convulsions, each lasting 
a very brief time, with loss of consciousness, frothing at the 
mouth, and jerky movements of the arms and legs. 

The Walker family was known to the police, since there 
were police records In two generations on the maternal side. 
The father was regarded as of rather low-grade mentality; 
a sister had committed suicide. Vivian herself had been 
irregular at school, was regarded as vicious, and had been 
hysterical. She had been committed to a reformatory at 
the age of 15 years. In the reformatory she had a number 
of excited outbreaks, with resentment of discipline, and these 
outbreaks presented hysterical traits. After each outbreak 
Vivian was depressed. It was during her stay at the re- 
formatory that her sister committed suicide. Vivian at- 
tended the funeral, and the idea of suicide appears to have 
taken hold of her mind, as she constantly spoke of suicide, 
threatened suicide, and made several attempts. She claimed 
at this time to see visions and to hear her sister's voice. 
On that ground she had been committed to a hospital for 
the insane at 16. 

At the hospital there were many fluctuations in mental 
condition. Vivian professed discouragement on account of 
poor home influences, telling how her mother had often been 
in prison, allowing Vivian to come under the influence of 
bad girls. Now and then Vivian had outbreaks of pro- 
fanity and glass-breaking, and she also made at the hospital 
for the Insane several half-hearted attempts at suicide. At 
the age of 19 she was returned to the reformatory, whence 
she was placed out on probation and allowed to return home. 

However, she was shortly re-commltted to the insane 
hospital in a phase of excitement, talking continuously of 
men and sex relations, and also of Imaginary illicit sex re- 



MEDICOLEGAL AND SOCIAL 30I 

lations with any man whom she happened to see. Again 
from time to time she made attempts at suicide. However, 
she was allowed to go out on visit, returned to her habits, 
and at the time of her arrest was living as a prostitute. 

After her convulsions in jail, she was admitted to the 
Psychopathic Hospital. At first obstinate and stubborn, 
later she became tractable. Special mental tests left her 
in the subnormal class, but we could hardly class her as 
feebleminded. We were able to observe her in a number 
of seizures, during which she would drop to the floor, ap- 
parently lose consciousness, writhe about, and assume the 
position of opisthotonos, the whole attack lasting but a 
minute or two. 

There was pelvic tenderness, with gonococci in the urethral 
smear. Salpingectomy had to be performed, but after the 
operation Vivian insisted upon getting up and running about 
on the second day, tearing the bandages from her abdomen, 
and infecting the wound. Outbreaks of excitement also 
followed the operation. 

In the diagnosis of this case, we must probably separate 
the convulsive phase from the remainder of the phenomena. 
The conduct disturbance, emotional outbreaks, and suicidal 
attempts date from early youth, and no doubt the diagnosis 
defective delinquent would fit Vivian from the beginning. 
The hereditary taint is characteristic enough. The sundry 
phenomena in the insane hospital, and particularly the hal- 
lucinations, lead one to wonder whether Vivian is not pos- 
sibly even suffering from dementia praecox. 

As to the convulsions, it would hardly appear that they 
are typically epileptic, although certainly epileptoid. Their 
onset at 22 is somewhat unusual. Several features of the 
seizures together with the opisthotonos and the previous 
history of hysteria, lead one to think of making the diag- 
nosis hysteria. 

I. Can cerebrospinal syphilis cause the symptoms? We 
found the serum W. R. to be positive though Vivian 
denied syphilitic infection. (She also denied gonor- 
rhoeal infection despite the clinical and laboratory 
findings.) We found that the spinal fluid yielded a 



302 MEDICOLEGAL AND SOCIAL 

gold reaction of a typical syphilitic nature, showed an 
excess of albumin, a slight amount of globulin, and 
130 cells per cmm. Even these findings, however, 
would perhaps not justify stating that the convulsive 
seizures are of syphilitic nature. The seizures dis- 
appeared under the administration of antisyphilitic 
remedies. It would seem, therefore, that the seizures 
should be regarded as of syphilitic nature. In any 
event, the diagnosis of cerebrospinal syphilis is justifi- 
able. This syphilis, however, is of an active nature 
and probably of recent production. We should be at 
a loss to explain the earlier mental features in Vivian 
as syphilitic and are therefore fain to associate the two 
psychoses. Psychopathic Personality and Diffuse 
Cerebrospinal Syphilis. 



MEDICOLEGAL AND SOCIAL 303 



NEUROSYPHILIS (*' paresis sine paresi ") in an 
habitual criminal, a forger. 



Case 88.* was brought to the hospital by the 

police. He was charged with having forged a check, and on 
account of the crudeness of the work his mental condition 
was suspected. 

Family History. The paternal grandfather was considered 
fast, drank a great deal and was said to be a thief. The 
father is said to have been forced to leave the State when a 
young man in order to avoid the reformatory. Paternal 
cousin murdered a man ; the sisters of this cousin said to have 
been wild and one brother married a prostitute. Nothing 
known of maternal relatives. 

Past History. Medical history is unimportant. He denies 
syphilis. His early childhood is of little significance. He 
was somewhat dull in school. At about the age of twelve he 
began to lie and steal, and has continued this ever since. 
His attempts have all been very crude, it is said, and when 
confronted he would strenuously deny his deeds, even when 
the evidence was overwhelming. He forged checks, borrowed 
money from all his friends, and charged things at stores to 
the family. The family paid the bills for a time, and then 
later had him sent to a reform school. He was married at 
nineteen, but wife has left him and obtained a divorce. He 
has been excessively alcoholic for years, and is suspected also 
of taking drugs. He was discharged from the navy dishon- 
orably. He later joined the army and was discharged there- 
from on account of " rheumatism," according to his account, 
but In reality deserted. He had finished a jail sentence of 
thirteen months for forgery a little over a year before entrance. 

Physical examination shows a well-developed and nour- 
ished man. The general physical examination Is negative. 

* Reprinted from article by Southard and Solomon : " Latent 
Neurosyphilis, the Question of Paresis sine paresi,'' Boston 
Medical and Surgical Journal, XXIV, i. 



304 MEDICOLEGAL AND SOCIAL 

The lungs show nothing abnormal. The heart is not enlarged, 
there are no murmurs or irregularities; blood pressure, 145 
systolic. The alimentary system is negative. No palpable 
lymph glands. Neurological examination: pupils equal and 
react to light and accommodation. Extraocular movements 
well performed. Tongue projects in the median line, with no 
tremor. There is no evidence of facial paresis or weakness 
of the muscles. The biceps, triceps, knee-jerks and ankle- 
jerks are present and equal on the two sides. There is no 
Gordon, Babinski or Oppenheim; no ankle clonus. There is 
no tremor of the extended hands. No Romberg sign. There 
is a little difficulty in the finger- to- finger test. There is no 
sensory disturbance either subjective or objective. No ten- 
derness over nerve trunks. 

Mental examination shows nothing of a psychotic nature. 
Patient is well oriented; memory for remote and recent 
events is well preserved, school knowledge well retained, grasp 
on current events good ; no delusions or hallucinations elicited. 
Patient is not feeble-minded, according to the intelligence 
tests of Binet and Simon and Yerkes- Bridges, but shows poor 
attention and gives evidence of weakness in volitional spheres ; 
is very suggestible. 

To summarize the case, then, we have a man of thirty years 
of age who has shown criminalistic and anti-social tendencies 
since childhood, whose general physical and neurological ex- 
amination is negative (excepting the laboratory tests), whose 
mental examination shows no psychotic symptoms, and who 
seems not feeble-minded. In other words, with the excep- 
tion of the serological and chemical findings in the blood 
and cerebrospinal fluid, there is nothing to suggest that he is 
more than a " criminal type." 

Wassermann reaction in blood serum positive. 

Wassermann reaction in cerebrospinal fluid positive. Ex- 
amination of cerebrospinal fluid: globulin ++, albumin ++, 
cells 55 per cubic millimeter; large lymphocytes, 9.1 per 
cent; small lymphocytes, 90 per cent; plasma, 90 per cent. 
Gold sol reaction, 3321000000. 



MEDICOLEGAL AND SOCIAL 305 

Can the criminalistic tendencies be condoned in this 
case on the ground of neurosyphilis? As a matter of 
fact the delinquencies in this patient reach back to 
early childhood and as there is no evidence of con- 
genital syphilis it cannot be held that syphilis had any 
bearing in the causation of symptoms. Even were the 
delinquencies only of recent date it is doubtful if the 
court would take cognizance of the laboratory findings 
in the absence of definite mental symptoms. In this 
connection it may be stated that the court takes cog- 
nizance only of the acts of a patient at time of exami- 
nation, and not of the history or laboratory findings, 
in committing a person. We have had several patients 
who from history, physical signs and laboratory tests 
made the diagnosis of paretic neurosyphilis easy and 
yet who could not be committed because they were 
mentally clear at the time. Such patients may be of 
grave potential danger to themselves and families, and 
present numerous social problems. See case of Joseph 
Wilson (95). 



306 MEDICOLEGAL AND SOCIAL 



JUVENILE PARETIC NEUROSYPHILIS (" juve- 
nile paresis ") with initial trauma. 



Case 89. Margaret Tennyson was a small girl of six 
years, described as having been normal until run down by a 
double-runner sled about 13 months before her arrival at the 
hospital. The change was stated to be remarkable. " She 
was as unlike her own self as darkness and daylight." Once 
fat and sunny, talkative and demonstrative with her toys, now 
Margaret had become silent, sullen, worried, and of a violent 
temper, stubborn and unmanageable. It does not appear 
that the patient was seriously Injured by the double-runner, 
as she was able to walk a short distance home. Shortly, 
however, she began to have trouble with her feet (diagnosed 
at the time as flat-foot), and thereafter her whole character 
and disposition changed. Upon arrival at the hospital, the 
patient walked with a typical scissors gait of spastic para- 
plegia. 

Physical examination was very difficult through lack of 
cooperation and a screaming and kicking resistance upon 
every attempt. There was a suggestion of hydrocephalus 
in the protrusion of the forehead. The pupils reacted readily 
to light and accommodation. The knee-jerks were active, 
but there was otherwise no disorder of reflexes. The patient 
had great difficulty in getting up from the floor, and for the 
most part Insisted upon lying in ventral decubitus on the floor, 
crying when attempt was made to raise her. An attempt 
was made to test her by the BInet scale, by which she was 
found to rate at 2| years although a portion of this low- 
rating was thought to be due to a failure of cooperation. 

The family history threw little or no light upon the case. 
The parents were living and well; a brother of 16 years was 
at work in the market district; two of the other siblings are 
in the first and second grades at school and regarded as ex- 
ceptionally bright by their teachers. The fourth was the 
patient, Margaret; a fifth had died at 9 weeks of heart trouble; 




Juvenile paresis — spastic paraplegia. 5 years. 



MEDICOLEGAL AND SOCIAL 307 

the sixth, seventh, and eighth, of 3, i| years and 3 months 
respectively, appeared entirely well. There were no mis- 
carriages or stillbirths. 

The scissors gait and spasticity seem to point undoubtedly 
to organic disease of the nervous system, along with which 
the mental deterioration seemed to suggest an active pro- 
gressive involvement of the cerebrum. The history seemed 
to be convincing that the child was not an instance of con- 
genital feeblemindedness. 

A neurologist's clinical diagnosis would naturally be syphilis. 
In point of fact, this diagnosis was borne out by the laboratory 
tests, which showed a positive W. R. in the serum and spinal 
fluid, positive globulin, a slight excess of albumin, and a 
syphilitic gold sol reaction. 

I. What is the significance of the trauma in the case of 
Margaret Tennyson? The trauma seemed to the 
family the precipitating cause. We find cases of gen- 
eral paresis in adults very definitely following trauma, 
yet neurosyphilis, both in adults and in younger patients, 
mainly occurs without trauma. On the whole, in this 
case, it is perhaps safer to regard the trauma as mere 
coincidence. A sister older than Margaret was found 
upon examination to have a positive W. R. The other 
children could not be examined. 



308 MEDICOLEGAL AND SOCIAL 



Traumatic form of PARETIC NEUROSYPHILIS 
(" general paresis "). 



Case 90. The point about Joseph O'Hearn was his entire 
mental soundness up to the time of an injury at work, when 
he was blown through a double window in an explosion, 
badly bruising his head. Shortly after the accident, although 
not immediately, the patient began to show signs of mental 
disorder, doing very foolish things, losing his memory, and 
becoming unable to work. 

It was eight months after the explosion when O'Hearn, at 
the age of 36, was admitted to the hospital with general mental 
impairment. O'Hearn was confused and disoriented for 
time and place, although he seemed to understand that he 
was in a hospital. He was given to foolish laughter and a 
silly manner. There was considerable emotional disorder; 
judgment was clearly impaired, and memory was poor. 

Physically, there was little to be found except upon neuro- 
logical examination. The right knee-jerk was greater than 
the left ; the tongue and fingers showed marked tremor, there 
was a speech defect and writing disorder. 

On the whole, it seemed impossible not to make the diag- 
nosis General Paresis, especially in view of the laboratory 
tests, with positive W. R. in both serum and fluid, a " pa- 
retic " type of gold reaction, 59 cells per cmm., excess albumin, 
and a large amount of globulin. 

I. What is the relation of the trauma to the paresis? 
Trauma is regarded as a precipitating cause, and In- 
dustrial Accident Commissions have been known to 
allow damages in such cases. Mott believes that the 
symptoms of a post-traumatic paresis must not develop 
until after a week's interval of freedom from symptoms, 
since he believes that time is required to destroy or 
irritate the brain to the point of producing the paretic 
picture. Our data are in agreement with those of 
Mott. Mott also points out that gumma sometimes 
occurs at the site of the trauma. 



MEDICOLEGAL AND SOCIAL 309 



False claim for compensation in neurosyphilis. 



Case 91. The facts in the case of Levi Sussman can be 
brought out by the following extracts from a report to the 
Industrial Board: A claim was made to the Board that the 
symptoms had developed after a fall from a building, some 
nine months before hospital observation. No connection could 
be found between this accident and the Paretic Neuro- 
syphilis found. We introduce the case to emphasize the 
possibility that irrelevant accidents may be regarded by 
ignorant or unscrupulous persons as setting up a mental 
disorder for which damages are claimed. If symptoms are 
already in existence before the accident and are not especially 
increased thereafter, naturally no damages should be re- 
covered. Unscrupulous persons may falsify about the pre- 
traumatic history and claim the development of symptoms 
immediately after the accident. Such claims are beyond 
question to be viewed with the greatest suspicion. Some 
days or weeks should elapse before definite symptoms in 
post-traumatic paresis appear. Just how long an interval 
may elapse between trauma and paretic symptoms and shall 
entitle the case to be regarded as one of traumatic paresis, 
is perhaps a matter of doubt. It would seem, however, on 
general grounds that three months is the longest period in 
which the post-traumatic effects are likely to be delayed. 

The question of traumatic paresis is of great interest on 
account of the war. The great strain under which the men 
at the front live and the physical injury due to being 
" buried " is probably responsible for an increasing number 
of cases of neurosyphilis. Such at least is the impression 
of Canadian medical officers with whom we have spoken. 
See Section VI, Neurosyphilis and the War. 



3IO MEDICOLEGAL AND SOCIAL 



Traumatic exacerbation (?) in PARETIC NEURO- 
SYPHILIS (" general paresis ")• 



Case 92. The case of Joseph Larkin was of note from the 
point of view of the Industrial Accident Board. This Irish 
teamster was said to have been injured in his head two or 
three months before coming up for examination at the age of 
45. For a week Larkin had had frontal headaches, had been 
sleeping poorly, and had been somewhat worried. In fact, 
he had stopped work. The W. R. of the serum was positive 
and a diagnosis of Paresis could be made. The case did not 
come up for consideration by the Industrial Board until two 
years after his initial appearance. 

The physical examination showed irregular pupils, sluggish 
pupillary reactions, Achilles absent, swaying in the Romberg 
position, enlargement of the heart to the left, positive W. R. 
of the blood and of the spinal fluid. 

Mentally, the patient's orientation for place was poor 
and his memory defective. Emotionally he was depressed or 
apathetic and was apprehensive. His flow of thought was 
slow, and his insight into his condition poor. 

It is interesting that a variety of causes have been assigned 
in this case for the condition: such as, his work, anemia, 
unhygienic surroundings, and arteriosclerosis. 

This case is not a sharply-defined case of post-traumatic 
general paresis, since there had undoubtedly been a variety 
of mental changes before the accident. Accordingly, re- 
covery of damages to a full amount could hardly be expected 
as in certain cases in which the phenomena of paresis appear 
only after the trauma. 




Post-traumatic cranial gumma — developing 13 months after local injury of skull. 



MEDICOLEGAL AND SOCIAL 3II 



Trauma : syphilitic lesion of skull at site of injury. 



Case 93. The medicolegal interest of Richard Marshall 
is extreme, as may be seen from the following brief report by 
the Psychopathic Hospital to the Industrial Board. 

" As to the case of Richard Marshall, a patient under 
the provisions of the temporary care act from December 
I to December 10, inclusive, this case has proved un- 
usually interesting in that the patient has proved to be 
syphilitic by the Wassermann reaction of the blood. 
There is no evidence of syphilis in the examination of 
the cerebrospinal fluid. The X-ray examination of the 
skull, taken in connection with the Wassermann reac- 
tion of the blood, warrants the diagnosis of syphilitic 
osteitis of the skull at the site of the old injury. We 
regard his present condition as shown by the X-ray 
as a syphilitic bone condition predisposed to by the 
injury. We do not find that the patient has any fea- 
tures of traumatic neurosis. 

" Mentally, having an actual age of 30, patient grades 
at 1 1.2 years. It may be that patient has always been 
a moron. He has earned about $8.30 a week. 

" We regard the patient as deserving treatment and 
feel that responsible parties in the case would do well to 
have such treatment instituted." 

The principal symptom of which Marshall complained 
was headache chiefly felt in the region of the osteitis. 
There was marked sensitiveness to percussion in this area. 
It is of course difficult to decide whether the headache 
was entirely due to the gummatous lesions or whether the 
trauma had caused contusions of the brain as well. It is 
also possible that the dura underlying*this area was involved. 



312 MEDICOLEGAL AND SOCLAi 



OCCUPATION-NEUROSIS in a granite-cutter: 
SYPHILITIC NEURITIS? 



Case 94. David Fitzpatrick was a case referred to the 
Psychopathic Hospital by the Industrial Accident Board. 
He was a granite-cutter of 52 years of age, and had begun to 
complain of pain in the forearm, extending back from the 
elbow, about six months before admission. It seems that 
the patient had been growing progressively worse and had 
thought he would have to quit work because of difficulty in 
grasping the hammer. A physician had told him that he 
must stop his work at granite-cutting or else he would en- 
tirely lose the use of his arm. He was in point of fact laid 
off because of slackness of work and had been unable to get 
work again. The pain in the arm, however, had continued 
and at times was very severe. Sometimes the pain and the 
worry led to insomnia. Fitzpatrick wanted the insurance 
company to pay certain accumulated bills, and maintained 
that he would be able to do work at $15 a week if work could 
be found for him. The general situation in this case can be 
gathered from the following abstract from the report to the 
Industrial Accident Board. 

" Secretary Industrial Accident Board, 
"Dear Sir: 

'' In re David Fitzpatrick 
referred to us with a copy of an Impartial report filed 
by the Massachusetts General Hospital, — we concur 
with said impartial report that there is now no evidence 
of paralysis of the arm. We do not find that the 
positive Wassermann reaction, although it indicates a 
history of syphilis, has affected the patient other than 
possibly to have reduced his general mental capacity. 
Our special tests yielded a percentage of 62% of what 
a patient of his age and station should possess. There 
seems, however, to be no connection between this reduc- 
tion of mental capacity and the difficulty with the arm. 
We cannot connect the history of alcoholism with the 
arm trouble. 



MEDICOLEGAL AND SOCIAL 313 

"There is some evidence that other stone workers 
have at times shown such effects. 

"The patient's fairly circumstantial account of his 
difficulty seems to point to a degree of myalgia or mus- 
cular pain in the region of the forearm when held in a 
certain position and a feeling of numbness in the third 
and fourth fingers. Whether these phenomena are due 
to local pressure upon nerves in the upper part of the 
forearm due to neuritis, or whether we are dealing with 
a functional neuralgic phenomenon is a question. 

" We have applied some special tests for faradic 
sensibility to all the fingers of both hands and have 
found that the fingers of the right hand are still less 
sensitive than those of the left, particularly the thumb 
and the little finger. This test has not yet been ap- 
plied in a sufficiently large number of cases to prove 
any difficult point, nevertheless the findings are in line 
with the patient's own circumstantial account of former 
feelings of numbness in the third and fourth fingers of 
|the right hand. 

" Obviously, then, our opinion is that there is still to 
be found some effect of the disease, whatever it was, 
which caused the patient to knock off work. If we had 
more experience with such cases and more data with the 
new test which we have applied, we should perhaps be 
inclined to admit the diagnosis of occupation neuritis, 
and to suppose structural alterations in the nerve trunks 
corresponding with the location of the muscular pain 
and the anesthesia of fingers and the dulling of electric 
sense, but in the present stage of our experience, it 
is probably wiser to call the case one of occupation 
neurosis y 

It is clear that the W. R. in this case was of peculiar value 
in at least partially clearing up the findings, yet it must be 
remembered that it is a principle of the modern adminis- 
tration of industrial accident boards and similar organizations 
that it is the employer's lookout whether the employee has 
syphilis. Recovery can be made as if the injury were due 
wholly to an accident. It was not possible however def- 
initely to prove or disprove a relation of syphilis in the form 
of a syphilitic neuritis to the condition in this case. 

The special tests above referred to are the electric sensory 
threshold tests of E. G. Martin. 



314 MEDICOLEGAL AND SOCIAL 



Character change: neurosyphilis. 



Case 9$. Joseph Wilson offered a very serious social 
problem. He was the father of two children, and his wife 
was pregnant at the time of his admission to the Psycho- 
pathic Hospital. He was a husky-looking man of 33 years of 
age, but for the past four years he had been deteriorating in 
his work ; he had been drinking heavily, and finally had stolen 
to obtain money for liquor. It was on account of his alco- 
holism and delinquency, which were taken as an indication of 
change of character, that he was sent to the hospital. 

Examination on his arrival disclosed at once that there was 
more to the case than alcoholism, for the neurological ex- 
amination showed that the pupils were irregular, the right 
being larger than the left, both reacting sluggishly to light, 
and there was an inequality in the reaction of the two eyes, 
the left being better than the right. The tendon reflexes 
were exaggerated, with ankle clonus on both sides, more 
marked on the right. There was also a marked speech de- 
fect. Otherwise the physical examination showed nothing 
of importance. 

The W. R. of the blood and spinal fluid was strongly 
positive. The globulin test was strongly positive, the albu- 
min was markedly increased, there were 74 cells per cmm., 
and a gold sol reaction of the " paretic " type. 

A formal mental examination did not show very much of 
consequence; his memory showed no marked impairment, 
he was not deluded or hallucinated, and he had a pretty 
good insight into his failings. However, he was somewhat 
childish, and his irritability was quite marked. Were one 
to rely upon the mental signs alone, it is probable that a diag- 
nosis of chronic alcoholism with deterioration would be made; 
but in the presence of the physical findings and the laboratory 
tests, the diagnosis of neurosyphilis had to be given. It is 
obvious that, while the patient was suffering from a pro- 
gressive brain disease, and while he did show mental symp- 



MEDICOLEGAL AND SOCIAL 315 

toms, there was not sufficient ground on which to commit 
him, and therefore he had to be turned out into the com- 
munity. As a matter of fact, he was not prosecuted on ac- 
count of his theft, because, although legally responsible, it 
was felt that his disease was at the basis of the character 
change which had led him into difficulties. Further de- 
velopments of his relations with society had to be considered, 
however. It was possible to get him to discontinue the use 
of alcohol altogether, and for nearly a year he has taken no 
alcoholic liquor and has been self-supporting. However, his 
irritability has been very great, making it very difficult for 
his wife to live with him, and causing his sister to break off 
all relations with him. 

Here, then, is a man with a marked Character Change 
as the result of neurosyphilis, so that it is difficult for him 
to maintain the usual social relations. It does not seem 
possible to remove him from the community. 

I. May one speak of general paresis without mental symp- 
toms? If one considers general paresis a mental disease, 
of course it cannot exist without mental symptoms. 
However, if one considers the disease as a chronic 
syphilitic meningoencephalitis characterized by its 
pathological anatomy, then one may readily speak of 
general paresis although no real evidence of mental 
symptoms can be discovered. It would seem that we 
must take this attitude with our present conception 
of brain localization, for it is easy to conceive of a 
general paretic process affecting areas which do not 
definitely relate to psychic function. And further, such 
a process may exist but not be of such a grade as to 
cause mental symptoms. 



3l6 MEDICOLEGAL AND SOCIAL 



The neurosyphilitic's family shotild not be forgot- 
ten in diagnosis and treatment. 



Case 96. The Bornstein family is remarkable. Let us 
hang the story on Becky, the mother, an Austrian woman of 
43 years, who appears to have been perfectly well up to within 
a year. About a year ago, Mrs. Bornstein began to suffer 
from severe headaches, which were treated with apparent 
success by an osteopath: at all events, Mrs. Bornstein re- 
covered therefrom in about six months. However, two 
months later, she had a convulsion, with foaming at the 
mouth, blueness of face, and general muscular stiffening. 
The convulsion lasted for several minutes. Again, a fort- 
night before admission, the patient had five convulsions of 
an identical nature in a single night. 

Moreover, since the first convulsion, Mrs. Bornstein's 
mental condition has altered and become variable, so that at 
times she is excited, at times depressed. She would assert 
inaccurately that there was some one in the house, and that 
she had at different times committed crimes of a heinous 
nature. Now and then she would seem to see moving pic- 
tures. Her memory was poor and she seemed to believe 
that events of five or six years ago had just happened. 

The pupils were sluggish, the knee-jerks and ankle- jerks 
were absent, there was slight ataxia, and there was speech 
defect. The suspicion of neurosyphilis was so strong that it 
seemed surprising that the W. R. of the blood serum, even 
after repeated tests and after the provocative injection of 
salvarsan, proved negative. However, the spinal fluid yielded 
a positive W. R., and a gold sol reaction of the " paretic " 
type, together with 12 cells per cmm., and a marked increase 
of albumin, with positive globulin. It would seem warrant- 
able to make a diagnosis at least of syphilis of the nervous 
system in this case, but it Is a question whether we should be 
warranted in making the diagnosis general paresis. 

That the diagnosis is doubtful may perhaps be seen from 



MEDICOLEGAL AND SOCIAL 317 

the variety of diagnoses in the rest of the family. In the 
first place, Mrs. Bernstein's husband admits syphilitic infec- 
tion many years before. He states also that his wife after 
marriage showed signs of syphilis and received some treat- 
ment, although limited. It is stated also that the husband 
himself at this time has a positive W. R. and has stiff pupils 
and petit mal attacks. The oldest son, 22 years of age, is 
confined in an institution with juvenile paresis. The second 
son has recently died at the age of 20 years, receiving a diag- 
nosis of rupture of the aorta. A third son, 19 years of age, 
has the appearance of having achondroplasia, although the 
proportions of his limbs do not quite correspond with those 
of an achondroplast. The fourth son, 17 years of age, is 
suffering from carles of the spine. A fifth son, 14 years old, 
is neurotic and has the so-called Olympic forehead. The 
sixth and last son died shortly after birth of unknown cause. 



31 8 MEDICOLEGAL AND SOCIAL 



Neurosyphilitic's normal-looking family proved 
syphilitic. 



Case 97. Walter Heinmas was a draughtsman 33 years of 
age when he was brought to the Psychopathic Hospital suf- 
fering from mental disease. This was diagnosed as general 
paresis, both on account of the clinical symptomatology and 
on account of the laboratory findings. In fact, it was a case 
of the classical type with marked euphoria and grandiosity. 

As is the routine procedure at the Psychopathic Hospital, 
in the case of all syphilitic patients, the family was sent for. 
This consisted of the wife and two daughters, aged 9 and 7 
respectively. The patient denied any knowledge of a syphil- 
itic infection. The wife, also, gave no history of any primary, 
secondary, or tertiary symptoms; there had been no abor- 
tions, miscarriages, or stillbirths; both children had been 
born at term and had been entirely healthy. Examination 
showed that the mother had no signs referable to syphilis, and 
that both the children were mentally well endowed, with 
good physique and showing no stigmata of congenital syphilis. 
Still the W. R. of all three (the mother and the two children) 
was positive in the blood serum. These tests were repeated 
several times on the children, with and without Injections 
of salvarsan, and they remained consistently positive. 

1. Are these children to be considered congenital syphilitics 

despite the absence of stigmata or symptoms? We 
must consider these children as congenital syphilitics 
and candidates for the group frequently spoken of as 
syphilitis hereditaria tarda. 

2. What is the frequency of syphilitic involvement in the 

mate and children of paretics? In our series, we have 
found that about 15% of the marriages where one 
member develops paresis, result in sterility; that In 
18% abortions, miscarriages and stillbirths occur; 
and that in 15% positive W. R. is obtained. We have 
adopted the motto : " The families of paretics are the 
families of syphilitics." 



MEDICOLEGAL AND SOCIAL 319 



Neurosyphilis: question of marriage. 



Case 98. Mr. Jacobs' wife was admitted to the hospital 
with a diagnosis of general paresis. A few weeks after her 
admission, she died as a result of her disease. According to 
our routine, her husband and the children were examined for 
evidences of syphilis. 

Mr. Jacobs' blood serum was found on repeated tests 
to be positive. He resolutely denied any knowledge of a 
syphilitic involvement, but it was later learned from his 
brother that about two years before his marriage — that is, 
more than 25 years before we saw him — he had acquired 
syphilis and had had a very small amount of treatment. 

Mr. Jacobs' was put upon antisyphilitic treatment in the 
form of injections of .3 gram of salvarsan every two weeks 
with occasional intramuscular injections of mercury salicylate. 
After seven months of treatment, the blood serum still re- 
mained positive. At about this time, the patient came to us 
to ask about getting married again. He said that he was 
living with his sister, who kept telling him that he was the 
cause of his wife's death, and this was so unpleasant that he 
desired to start a home for himself again! 

1. What advice should be given? It is a general opinion 

that the longer the period after the initial infection, the 
less the chances of infecting a partner. This chance 
is further reduced under antisyphilitic treatment, of 
which a considerable amount had been given in the 
case of Mr. Jacobs. However, when one considers 
the trickiness of syphilis and the fact that there is some 
chance of infection, which we would apparently over- 
look if we gave him permission to marry at this time, 
the only possible course was to tell the patient that he 
should not consider marriage until his Wassermann 
had become negative and remained so for some time. 
The children in this case were negative. 

2. What is the physician's duty to the family of a syphilitic 

patient? It is our firm conviction that it is the duty of 



320 MEDICOLEGAL AND SOCIAL 

every physician to his syphilitic patient, to the patient's 
family, and to the community, to examine the mate 
and the children for evidence of syphilis acquired or 
congenital and to offer treatment if it is found to be 
needed. This is one of the chief means at our disposal 
today to prevent the late disasters of syphilis, acquired 
or congenital, for by such examinations the syphilitic 
condition is discovered before lesions have occurred 
which are irreparable. We know that the mate and 
children of a syphilitic patient have been exposed to 
syphilitic involvement, and it is our duty as physicians 
in possession of such knowledge, and as guardians of the 
public health, to investigate such cases, so that if they 
be found to have syphilis, steps may be taken to treat 
them early. 

3. How much danger is there of causing unhappiness and 

breaking up families by this procedure? This question 
offers a chance for many theoretical answers. The 
facts are, however, that in doing this as a routine 
for nearly three years and examining several hundred 
families, there has been no instance to our knowledge 
in which a family has been broken up or grave difficul- 
ties have been encountered by this procedure. 

4. In what percentage are the mates or children of neuro- 

syphilitics found to show definite symptoms of syphilis? 
It is our opinion that the situation in regard to neuro- 
syphilitics is the same as for syphilitics in general: 
That the same laws of attenuation of virus, and of 
chance occur here as elsewhere. 

Just as this book is going to press, we have learned that the 
distraught Mr. Jacobs, still desirous of starting a home for 
himself and feeling entirely well, consulted a physician. 
This physician took a sample of blood and had it tested at a 
competent laboratory, which reported the blood negative. 

On the strength of this test, the physician felt himself 
warranted in recommending, or at least not advising against, 
Mr. Jacobs' marriage, which has probably now taken place. 

Although there is some doubt what ethical relation a state 
institution shall maintain with physicians in private practice, 
we took occasion to call the attention of our patient's new 
counsellor to the fact of the patient's neurosyphilis. We 
noted that the man's serum had been constantly positive 



MEDICOLEGAL AND SOCIAL 32 1 

(Massachusetts State Board of Health findings) in a score or 
more of observations. We called attention to the fact that 
lumbar puncture had shown positive signs of neurosyphilis, 
including a positive W. R., globulin, excess albumin, pleocy- 
tosis, and positive gold sol. These facts, according to a let- 
ter received from the private practitioner in question, have 
not altered his opinion in the slightest to the effect that our 
patient is completely normal and entirely suitable for mar- 
riage. It is clear that he regards the patient as not a victim 
of General Paresis. 

5. What is the significance of the negative observation 
eventually obtained in Jacobs' serum? One's first 
thought is to impugn the accuracy of the laboratory 
work, but against this suspicion is the excellent repu- 
tation of the laboratory in question, and the agree- 
ment in the majority of its findings with those of the 
State Board of Health. It is more likely, as we assured 
the private practitioner at whose request the observa- 
tion was made, that this negative test was an exceptional 
and isolated observation such as is not infrequent in 
long series of observations, particularly those made 
under therapeutic conditions. In so important a mat- 
ter, we are inclined to feel that the physician in question 
should have resorted to two more observations at in- 
tervals before running counter to the position taken 
by the hospital. 



— many a hard assay 
Of dangers, and adversities, and pains. 



Paradise Regained, Book IV, lines 478-479. 



V. SOME RESULTS OF TREATMENT 

Cases 99-103 show the Variety of Structural Lesions that 
Treatment has to face. 



SPASTIC HEMIPLEGIA in PARETIC NEURO- 
SYPHILIS ('* general paresis"), showing marked 
degenerative changes, a condition in which therapy 
could be theoretically of very little avail. Autopsy. 



Case 99. James McDevitt arrived at the Danvers Hos- 
pital, July 20, 1906 (saying that he came to be " thawed 
out "), and died less than six months later: January 12, 1907. 
He was 34 years of age. He had been a shoe-worker after 
leaving school, had worked eight years with the General 
Electric Co., and had then become a bartender. He had, 
however, stopped work in September, 1905, and we may 
safely say that mental symptoms had begun insidiously at 
about that time. His symptoms, if there were any, had been 
masked by a heavy alcoholism, but an obvious change had 
appeared in November, 1905. The patient lost ambition, 
smoked and loafed about his room, and developed speech 
disorder. He denied venereal disease, nor was there any 
superficial evidence of such. 

Physically, the patient showed little or no disorder except 
acne of the trunk, patches of eczema on the left lower chest, 
and numerous brownish scars along both tibiae. 

Neurologically, the Romberg position was maintained, but 
the gait was very unsteady on attempts to walk a straight 
line; fingers, tongue, and face were tremulous, and finer 
movements were performed with marked incoordination. 
No direct or consensual light reactions could be obtained In 
the pupils, which were dilated and irregular. 

The condition of the reflexes is important on account of 
the autopsy findings. The abdominal and cremasteric re- 
flexes were prompt, and the knee-jerks equal and very lively. 

323 



324 TREATMENT 



COMMON THERAPEUTIC CONCEPTION 

[M]VP = TYPICAL PARESIS 

MV[P] = TYPICAL CEREBROSPINAL SYPHILIS 

[M]V[P] = TYPICAL SYPHILITIC ARTERIOSCLEROSIS 



(M = Membranes, V = Vessels, 
P = Parenchyma, [ ] = not involved) 



Chart 21 



TREATMENT 325 

Achilles and normal plantar reactions were present; there 
was no clonus ; the arm reflexes were very brisk. 

The mental symptoms need not detain us. Consciousness 
was clear ; orientation for time, place, and to some extent for 
persons, was imperfect. Arithmetic had been largely for- 
gotten. Handwriting was irregular and scrawling, and in 
places unintelligible. Although the patient claimed that 
his memory was intact, it was decidedly imperfect. He 
remarked that John D. Rockefeller, a Chicago king, was Presi- 
dent; the General Electric Works had almost 50,000 people 
at work; and in fact Lynn was one of the largest cities in the 
state, having over 12,000 people. The height of patient's 
room was estimated at 25 feet. There was a slight eupho- 
ria. There was never any doubt of the diagnosis of Paretic 
Neurosyphilis ("general paresis"). 

Five months after admission, slight convulsions developed, 
after which the patient was more dull and demented; he 
became bedridden. More convulsions followed, leaving the 
right arm and hand useless. There were clonic spasms of the 
muscles of both lower legs. Decubitus developed and death 
occurred. 

We may set the total duration of symptoms in the case of 
James McDevitt at a little over a year; nor is there any 
evidence of previous or prodromal symptoms beyond a total 
period of about 15 months, unless we may regard his leaving 
the General Electric Works to become a bartender some nine 
years before death, as a symptomatic change of character. 
In any event, it is of note that the autopsy showed singularly 
few lesions. Death was due doubtless to complications 
following decubitus, and there was a slight acute splenitis. 
The kidneys showed some parenchymal change. The aorta 
showed many patches of sclerosis, with calcification or ulcera- 
tion throughout its length. These changes were not charac- 
teristic of syphilitic disease. There was considerable coronary 
arteriosclerosis and a slight mitral valvular sclerosis. There 
was a brown atrophy of the heart muscle, somewhat surpris- 
ing in a man of 34 years. The brain was practically normal, 
weighed 1200 grams, and showed convolutions normal in 
size, relation, and arrangement. There was no sclerosis 



326 TREATMENT 

grossly evident in the blood vessels. The pia mater appeared 
to contain a considerable excess of clear fluid. The calvarium 
was of normal thickness and showed diploe and the dura 
mater failed to show adhesions. There were no macroscopic 
signs of lesion in the spinal cord. 

Microscopically, the lymphocytosis, plasmocytosis, and 
phagocytosis of the perivascular spaces, (relative?) increase 
in blood vessels, the gliosis, and evidence of nerve-cell destruc- 
tion, taken together warranted the diagnosis of Paretic 
Neurosyphilis. It was plain that the nerve-cell destruction 
was best marked in the inner layers of the cortex. The 
microscopic study of the spinal cord showed that there was 
very possibly a slight sclerosis of the posterior columns in the 
lumbar region, but this was so slight that it could hardly be 
noted in the myelin sheath stains (Weigert). Very sharply 
marked, on the other hand, were the bilateral pyramidal 
tract lesions in the lumbar and thoracic regions, less marked 
at the cervical levels. 

Without attempting to analyze carefully all these findings, 
it is interesting to note in this case a foil to the usual spinal 
cord picture of paretic neurosyphilis. The spinal cord, ordi- 
narily normal, or perhaps more usually affected by a degree 
of posterior column sclerosis, in this case showed such well- 
marked pyramidal tract sclerosis that we may perhaps place 
the case in a subordinate group of Spastic Paretic cases 
of Neurosyphilis. The source of the pyramidal tract dis- 
ease lodges, however, in the cortex cerebri itself, being part 
and parcel of the lesions mentioned above as affecting more 
directly the inner layers of the cortex. Many of the so-called 
giant, or Betz, cells had undergone a complete destruction. 
It will be remembered that clonic spasms of the muscles of 
the legs appeared in the fortnight preceding death, and that 
there had been convulsions for about six weeks before death. 
There was no evidence at the autopsy why the right arm and 
hand should have become useless, whereas the left upper 
extremity remained normal. This case, then, forms an ex- 
ception to the ordinary paretic neurosyphilis group in that 
the brunt of the microscopic process was borne by the inner 
layers of the cortex. The cells of origin of the pyramidal 




Bilateral pyramidal tract sclerosis, secondary to destruction of large motor (Betz) 
cells of motor (precentral) cerebral cortex — paretic neurosyphilis. 



TREATMENT 32/ 

tract fibres had been cut in this lesion, and had become sub- 
ject to partial or complete destruction. Note, however, 
that the lesion remained a microscopic one and that the 
marked convulsions were not related to gross lesions, thereby 
following the rule for paretic seizures. 

From the standpoint of possible treatment, It is of course 
true that few organs of the body showed grave lesions save in 
the calcified and ulcerated aorta, which conceivably might 
have become quiescent under appropriate treatment. But, 
although the brain was almost if not quite normal in the gross, 
and although its membranes showed practically no lesion, 
treatment would not have been very promising. To be sure, 
the exudate might have been cleared away if the spirochetes 
responsible therefor had been destroyed by treatment. Yet 
the destruction of the giant cells of origin of the pyramidal 
tract fibres to such an extent as in this case could hardly 
have been compensated for by any known process. So far 
as we are aware, the destruction of considerable numbers of 
the smaller association elements of the brain is subject to the 
compensation of other elements of the nervous system, which 
conceivably might be re-educated or newly educated to per- 
form certain processes. The histological picture in a case 
like that of McDevitt accordingly leads to the hypothesis 
that so well marked a spastic paresis, even in the presence 
of otherwise favorable signs, would be of especially baneful 
portent therapeutically. 



328 TREATMENT 



NEUROSYPHILIS with total duration of symp- 
toms twenty-two days. The comparatively MILD 
BRAIN LESIONS, INFLAMMATORY AND NOT DE- 
GENERATIVE in type, suggest the possibility that 
therapy might have been successful. Autopsy. 



Case 100. Jacob Methuen, 35, was a carpenter from 
Newfoundland. He was working upon a certain Thursday 
with his brother, who noticed that Jacob was Ufting the tools 
about in an unusual manner and talking strangely to his 
fellow workmen. He fell asleep, going home in the street car, 
and said afterward that he felt dazed and peculiar. He talked 
all kinds of nonsense to his wife upon arrival. Methuen 
remained in bed next day, fancying he was going to die, 
calling his family together, and saying good-bye to them. 
He remained in bed all through the next day, but on Sunday 
appeared better, — more active, and in fact quite natural. 
He slept only an hour Sunday night, calling to his wife that it 
was time to get up. On Monday he began to be irritable 
to his wife, and accused her of flirting with his brother and 
intending to elope with him. He struck his wife several 
times, and when two brothers came to watch him, accused 
them both of trying to steal his wife, and struck them. Tues- 
day he remained in bed until late at night, when he arose 
and tried to assault the family. 

It seems that another brother of the patient had died but 
eleven days before his admission to the hospital and five days 
before the onset of Jacob's symptoms. Since his brother's 
death he had been dwelling upon religious matters, and in 
fact the day after his brother's death, he waked up during 
the night, saying that he was too happy to sleep, that he 
heard the Master's voice, and at times the devil's voice; 
that there was to be a modern miracle and his spiritual life 
from now on would be different. 

Eleven days after admission to the hospital, Methuen 
died, making a total duration of symptoms, beginning at his 
brother's death, of 22 days. 



TREATMENT 



329 



NEUROSYPHILITIC LESIONS 

LESIONS OF THE SECONDARY PERIOD 

(i) INTERSTITIAL ENCEPHALITIS OR MYELITIS 
("meningitis") 

(2) PARENCHYMATOUS ENCEPHALITIS OR MYELITIS 

(" encephalitis, " "myelitis ") 

LESIONS OF THE TERTIARY PERIOD 

(i) CHRONIC INTERSTITIAL ENCEPHALITIS OR MYELITIS 
("gummatous meningitis ") 

(2) CHRONIC PARENCHYMATOUS ENCEPHALITIS 

(" dementia paralytica ") 

(3) CHRONIC PARENCHYMATOUS MYELITIS 

(" tabes dorsalis") 



" We have shown that the central nervous system is affected by syphilis 
at the same periods and in the same manner as are other internal organs. 
In addition the ' parasyphilitic ' lesions are also of a typically syphi- 
litic nature, being directly comparable to the parenchymatous affections 
found elsewhere in the body. They are 'tertiary' lesions differing 
only from the so-called 'gummatous' processes in the central nervous 
system in that their localization is in the parenchyma while that of the 
latter is in the interstitial tissues. " 

McIntosh and Fildes, 1914 



Chart 22 



330 TREATMENT 

Physical examination showed a man 5' 9" tall, weighing 
149 pounds, rather pale and poorly nourished, with a some- 
what enlarged heart and no evidence of venereal disease, 

Neurologically there was a slight facial and digital tremor, 
but otherAvise no symptom or reflex disorder except that the 
tendon reflexes were generally increased; the knee-jerks 
especially were very vigorous. There was no speech defect. 
His handwriting was fairly legible. 

The patient was very noisy and uncontrollable, tearing 
clothing and biting, striking the attendants, refusing food, 
talking rapidly, loudly, and incoherently. His manner sug- 
gested auditory hallucinations but no positive evidence of 
these was obtained. His clothes could not be kept on him. 
The following is a sample of his reactions: As the examiner 
entered, the patient stood stark naked and glaring. He 
started to talk as follows: " Methuen, — I, Saviour, come 

to life and ought to die Now I lay me Now I 

die The heart beats No, I ain't going to die 

■ I am going out soon. I want my clothes You 

can't hold me; I am strong." (Struggles violently with the 
attendants.) " I am God. God. I know you, you can't 

fool me. I am here I can do you all. How 

many doctors are there here? " (Struggles violently. Looks 
at examiner.) " He is writing something. Sir, you can't fool 
me in a million years. Do you understand that, doctor? 
You can't fool me. Write all the prescriptions you want to. 
Ten thousand years; you hear that, doctor? Ten thousand 
years. You can't fool me; ten thousand years. Ten thou- 
sand years are but a day for the spirit of the Lord," etc., etc. 

The excitement continued unabated. The patient became 
entirely disoriented, and finally almost unable to move. 
He lay in bed trying to talk and muttering broken gibberish, 
still attempting to struggle to the extent of his limited 
strength. 

The autopsy showed no sign of lesion (brain weight 1380 
grams), unless, perhaps, the occipital regions were slightly 
firmer than the rest of the brain. Death was apparently 
due to a bilateral pneumonia, bronchial type. There was 
an acute splenitis. The only chronic lesions of the body 




Paretic neurosyphilis ("general paresis" ) macroscopically normal, mi- 
croscopically characteristic. Treatment does not have to face massive 
destructive processes already complete. 



TREATMENT 33 1 

were a bilateral chronic adhesive pleurltis and a slight scler- 
osis of the arch of the aorta. 

Microscopically there was a distinct though mild degree 
of lymphocytosis of the perivascular spaces In many regions. 
Somewhat extended search failed to reveal plasma cells, and 
it is certain that if plasma cells existed, they must have 
occurred In very small numbers. 

Here, then, was a case of Diffuse Neurosyphilis (with 
brain picture consistent) with symptoms lasting but 22 days 
and with an appearance of acute mania. It is to be noted 
that this case arrived at the hospital on the eleventh day of 
his symptoms. The case occurred long before the develop- 
ment of the temporary care system in Massachusetts. It is 
probable, or at any rate possible, that he would have been 
brought to the hospital far earlier, say, upon the sixth day, 
had the modern temporary care system been installed at 
that time. The routine W. R. examination would then 
have been made. With more effective hydrotherapy. It is 
possible that the patient's life might have been prolonged 
and that treatment might have been effective. So far as 
we can see, the case would have been a singularly good one 
for treatment despite the practical unmanageabllity of the 
case under ordinary home treatment, and even under hos- 
pital conditions where modern hydrotherapeutic appliances 
are not available. 



332 TREATMENT 



PARETIC NEUROSYPHILIS showing very 
MARKED MENINGITIS, suggesting that therapy 
might have produced improvement. Autopsy. 



Case loi. We report the case of John Baxter, a boat 
tender of 48 years, because this particular victim of Paretic 
Neurosyphilis seems to have had the most markedly thick- 
ened and altered meninges in our whole series. Of course, the 
therapeutic theory upon which we now proceed in the treat- 
ment of non-paretic and possibly even of paretic neuro- 
syphilis is that, other things being equal, the meningitis 
can be removed by treatment, or in the course of treatment, 
so that the degree of ultimate recovery rather depends upon 
the condition of the brain substance itself than upon the 
condition of the meninges. Here, at all events, is an example 
of the most highly meningitic neurosyphilis that we have 
seen. 

Curiously enough, two of Baxter's brothers were also 
patients at the hospital at which Baxter died, and a number 
of the other members of the family are reported as "nervous." 
It seems that at 35 Baxter began to drink heavily and had 
never given over the habit of alcoholism. 

Upon admission to the hospital, in fact, he showed a suf- 
ficiently typical picture of delirium tremens. His conscious- 
ness was clouded, he had vivid visual hallucinations and was 
very apprehensive. 

His heart was enlarged to the left; the pulse, 120, was of 
increased tension and irregular; there was peripheral arterio- 
sclerosis; the teeth were poor; the tongue coated; and 
the mouth foul. The urine showed a trace of albumin and 
rare hyalin casts. 

NeurologicaUy, the gait was somewhat unsteady, there 
was an extreme tremor of the whole body, including the 
tongue and fingers. The Romberg sign was negative although 
there was marked swaying. The pupils were equal and reacted 
normally; the knee-jerks were markedly exaggerated, the 




A high degree of chronic leptomeningitis. Pia mater thick, opaque, 
concealing brain. In paretic neurosyphilis ("general paresis")- 



ii 



TREATMENT 333 

arm reflexes somewhat exaggerated. The remainder of the 
reflexes upon systematic examination were negative. 

Upon arrival, Baxter was put to bed, but he barricaded 
his door and fought with the attendants. The tremor in- 
creased, the hallucinations were both visual and auditory. 
After a few days, Baxter became so weak that he could not 
move. He refused to eat for a period of two days, explain- 
ing in whispers that he did not wish to be poisoned ; a voice 
had told him the food was to be poisoned. The voice was of 
agreeable tones, probably belonging to a lady; it did not 
speak, but sang to him. The clouding of consciousness 
failed to clear up, as in delirium tremens, so that, though 
patient was admitted March 3d, it was hardly possible to 
speak freely with him until more than a month later, April 9th. 
A goodnatured conversation would run as follows * 

"What is your name?" " Baxter." 

"First name?" After long pause, "Don't know." 

"John?" Pause of 7 seconds, "Yes, I 

think it is." 

"How old are you?" " There are legs there is a 

body up to here ' ' 

"Say the alphabet." Term not understood. 

"Say the a, &, c." "Oh yes; a, b, c, d (long 

pause), e, f ; I cannot say it, 
I did not have much educa- 
tion; I am not intelligent." 
(In point of fact, the patient 
had a good grammar-school 
education, and had long 
worked as a clerk in a gro- 
cery store, with good wages.) 
There was some speech defect. 

Soon the hallucinatory phase passed, and the patient 
remained in a cloudy and disoriented state, inaccessible, 
rarely speaking, and gradually failing physically. Death 
occurred about three months after admission (pulmonary 
symptoms) . 

In estimating the duration of the process in John Baxter, 
we must take into account that he left the grocery business 
and became a hard-working but poorly-paid boat tender at 



33.4 



TREATMENT 



about 35 years, at the same time that the alcoholic llabit 
began. 

The autopsy showed that death was due to bronchopneu- 
monia with pleurisy. There were in the body a variety 
of chronic lesions, such as gastritis, colitis, epididymitis, 
splenitis, parietal and valvular endocarditis, prostatitis, chronic 
appendicitis, and some mesenteric lymphnoditis. The heart 
was somewhat hypertrophied. There was a slight diffuse 
nephritis with cysts, emaciation, and decubitus. The cal- 
varium was thick and somewhat dense. The dura was 
thickened and adherent, and the pia mater, — as above 
stated, the most thickened and altered pia mater in our 
series, — is described as everywhere thickened, of a brownish 
gray and white color, especially over the vascular lines, and 
as showing small white areas of deeper thickening scattered 
over the surface, but most markedly over the sulci, and not 
as a rule over the crowns of the gyri. There were also yellow- 
ish brown spots with a suggestion of fibrin over the lateral 
aspects of both hemispheres. The vessels at the base were 
not remarkable in the gross. The brain weighed 1220 grams, 
and appeared to be of darker color than usual. 



TREATMENT 335 



Some cases of PARETIC NEUROSYPHILIS 
(" general paresis ") have so much BRAIN 
ATROPHY that it is not possible to expect much 
improvement through antis3rphilitic therapy. 



Case 102. Theodosia Jewett, dead at 58 years, showed the 
most remarkably wasted brain in a long series of victims of 
paretic neurosyphilis. We present her case to emphasize 
what therapy must face in certain instances, but would 
recall the fact that exceedingly few such wasted brains have 
come to our attention in cases dying in the institutions of 
Massachusetts. 

Mrs. Jewett, a housewife, whose parents died of shock, and 
one of whose two brothers also died of shock, was a normal 
child and schoolgirl, and worked as dressmaker until she 
was married, at 24, to a grocer, by whom she had two children. 
At the age of 46, Mrs. Jewett began to suffer from so-called 
" nervous prostration." The attack lasted some two years, 
but there were no psychotic symptoms beyond worry and 
insomnia. The menopause occurred at 52, at which time 
the first signs of psychosis appeared, namely, a forgetfulness 
concerning familiar matters, such as sewing, cooking, and 
the like. At 55, this amnesia had become so marked that 
Mrs. Jewett could neither write nor tell time. She, however, 
was a perfectly quiet and easily manageable patient, often 
subject to drowziness in the day. 

Six months before her admission to the hospital, she began 
to suffer from insomnia, failed to recognize her surroundings, 
and had a number of crying spells. Restlessness had begun 
a month before admission ; auditory hallucinations developed 
in the form of imaginary conversations with dead persons. 
A certain loquacity set In, and for a week before admission, 
Mrs, Jewett became somewhat resistive. 

Physically, the patient was sallow, poorly nourished, with 
pale mucous membranes, peripheral arteriosclerosis, no teeth, 
muscular feebleness, tremor of hands and tongue, and active 



336 TREATMENT 

knee-jerks. Mentally, the patient was depressed, talked to 
herself, assumed a suppHcating position, suddenly altered 
her attitude, and was very tremulous. Her talk was low, 
mumbling, and incoherent, for the most part composed of 
answers to her own questions. Sometimes there was a 
curious difficulty in speaking, such that the lips moved but no 
sound emerged ; but for the most part there was no difficulty 
in uttering words. The patient either could or would not 
write. Only when the attention was secured by speaking to 
her sharply was she apparently able to understand questions, 
and the answers to these sharp questions came spasmodically 
and as if interrrupting her own thoughts. Nor was it ever 
possible to obtain a repetition of the same answer. 

The patient died in exhaustion, with pulmonary symptoms 
three weeks after admission. 

The autopsy which was performed 3I hours after death 
showed the following points of interest: 

The heart weighed 210 grams. There was marked thick- 
ening of the aortic valve. The coronaries were slightly 
thickened. 

The lungs were slightly adherent to the chest wall at the 
apices and posteriorly. The right lung was consolidated 
in the lower two lobes posteriorly and the bronchi exuded 
pus ; the left lung was not remarkable. There was a chronic 
splenitis. 

The liver showed fibrous changes, was a brownish-red in 
color, mottled with yellow. 

Combined weight of the kidneys 195 grams. The capsules 
were adherent, tearing the cortex when stripped. 

The diploe were well marked. The dura was not adherent. 
The pia was slightly thickened and raised from the cortex by 
a large amount of subpial fluid (showing atrophy of the cortex). 
The pial vessels were injected, more markedly so on the left 
side. The arachnoid villi were reported as moderately de- 
veloped, especially along the longitudinal fissure. 

The brain was rather soft in all regions. The weight 
was 1045 grams. According to Tigges' formula the weight 
of the brain should be approximately 8 times the body length 
in centimeters. The length in this case was 1 58 cm. , therefore, 






;^'•A'^,*!:^C:"^:^::^■'^•• 



cA 






'J ..--. 







■ .,k_::'-Ul 



."J-' ■^'' 









^.■; I. 



,'|^';;^-.;^./,./- 



i: v.. 



J . \ ' 



«i ■; .- /i' ''i' •'■4 •« '/ 



:^- --' -.\ ■-.. .r -^ ,. ^ ' * /v. 'i- > v^*^ ■•,-.<, .,'^ , 



/'.v 






*v\ "'^^ 



Perivascular exudate (low power) 
in atrophic cortex from case of gen- 
eral paresis. 



Markedly atrophic cortex, but with- 
out local perivascular exudate. 



TREATMENT ' 337 

according to this formula the weight of the brain should have 
been 1464 grams. The difference of more than 400 grams 
is evidently a loss to be accounted for by atrophy, a very 
heavy loss. 

1. Was the " nervous prostration " at 46 of syphilitic 

origin? One cannot give a categorical answer to this 
question. The high incidence of shock in the family 
suggests poor stock in which a psychoneurosis is not 
an unusual phenomenon. The presence of syphilis 
might act as a debilitating factor or agent provocateur, 
if it were not to cause any demonstrable brain lesion. 
As pointed out in the case of Harrison (9), however, 
it is not unusual in neurosyphilis to find a history of 
symptoms occurring years before the final breakdown 
and symptoms frequently not recognized as of neuro- 
syphllitic nature. 

2. Does the fairly long duration of the psychosis (at least 

3 years) explain the marked atrophy? Cases having 
symptoms even much longer than three years at times 
show relatively very little atrophy, so that this factor 
in itself cannot be said to explain the tremendous 
destruction of tissue. 



338 TREATMENT 



The THERAPY OF NEUROSYPHILIS has to face 
not merely variations in the degree of brain 
wasting and in the degree of meningitis, but also 
variations in the topographical distribution of 
lesions. Autopsy. 



Case 103. To bring out this point we may instance, the 
case of Alfred Weed, a victim of Paretic Neurosyphilis, 
dying at the age of 48 years after a course of about seven 
years. The following is an abstract of the clinical history: 

A. W. suffered from lues some 24 years before his death at 
Dan vers Insane Hospital in 1907. There is no account of 
insanity in his family. The patient had been undergoing 
mental changes for six years before death. At the age of 42 
began to take interest in socialism and spiritualism. Would 
become excited at times and was observed to talk to himself. 
At times it seemed that he was reacting to visual hallucina- 
tions. After eight months he became depressed and appre- 
hensive and developed delusions of poisoning. 

On admission to the Danvers Insane Hospital in June, 
1902, the subject was found to be ataxic, falling in the Rom- 
berg position. Pupils were equal but of pin-point size. 
There was tremor of the facial muscles. The knee-jerks were 
absent. Speech was ataxic. Memory defective. Depressed. 
Thought he was to be punished. Refused to eat. 

Later in the year of admission, patient became more 
negativistic. He refused to have his clothes brushed. His 
answers were slow. Mental arithmetic was correctly but 
slowly done. During January, 1903, the patient was apt to 
be active and talkative for a time, and then his attitude 
would suddenly change to one of silence, resistivement and 
untidiness. From time to time he would be querulous 
and sulky. In August, 1903, the patient became weaker 
and could walk with assistance only. Paralysis developed 
in the left facialis region and In the left external rectus. 
Pupils were still small, but the left had become smaller than 



TREATMENT 339 

the right. Light reaction tests unsatisfactory. Knee-jerks 
could not be obtained. 

In December, 1903, the patient was untidy and helpless, 
lying with his thighs and legs flexed. The limbs were spastic 
on passive motion. In 1905, the pain sense of the legs was 
found lost and the pupils were small and stiff. The pro- 
truded tongue was deflected to the right. The right labial 
fold was more prominent than the left. Knee-jerks remained 
absent. Ataxia was extreme. 

The Neurological Findings may be summed up as follows: 

1. Ataxia of the legs. 

2 . (Probable) Diminished sensibility in the legs. 

3. Pupils small and stiff. Left smaller than 

the right. 

4. Paralysis of left facialis. 

5. Paralysis of left external rectus. 

6. Tongue protruded to right. 

7. Right elbow jerk greater than left. 

8. Knee-jerks absent. 

The cause of death was bronchopneumonia. The walls 
and valves of the heart showed a few chronic changes. There 
was a marked splenitis and an atrophy of the liver. The 
kidneys showed numerous depressed scars. The arch of the 
aorta was somewhat sclerotic. The following is a full de- 
scription of the head findings which we present by way of 
comparison with other cases. Note especially the cerebellar, 
dentate, and olivary changes. Note also the fact that pal- 
pable sclerosis is demonstrable over a far larger area than 
atrophy, so that we may almost safely conclude that the proc- 
ess of induration sometimes precedes that of atrophy. One 
gets the impression from the extent of visible atrophy and 
tangible induration in this case, that a possible therapy would 
have not merely to clear the perivascular spaces of cells and 
spirochetes, but would also need to arrest the indurating and 
wasting process. Nor could any therapy deal effectively with 
the superior frontal and upper central atrophy of the cerebrum 
of this case, or with the olivary and cerebellar lesions. 

Head: Hair thin at vertex. Scalp normal. Calvarium 



340 TREATMENT 

thin and dense. Dura mater slightly adherent to calvarium 
at vertex. Sinuses normal. Arachnoidal villi well devel- 
oped. Pia mater of anterior and central regions contains an 
excess of fluid. The pial veins well injected. 

The pia mater exhibits one unusual lesion : Faintly yellow- 
ish brown spots of miliary and slightly larger size are scat- 
tered irregularly in clusters over the vertex. These miliary 
pial macules are observed especially over the posterior third 
of the left superior frontal gyrus (a group of twelve or more). 
Two are seen in the pia mater of the right superior frontal 
gyrus. One is seen in the upper part of the left post central 
gyrus. The upper end of the right postcentral gyrus con- 
tains three macules. 

Besides these brownish macules, the pia mater also shows 
focal white thickenings which resemble the more frequent 
appearances of chronic fibrous leptomeningitis. The white 
thickenings are of irregular size but are, as a rule, larger than 
the macules above mentioned. They occur, as a rule, over 
the vsulcal veins and are most frequent in the anterior region. 

The vessels at the base are normal. There is no evidence 
of pial thickening at the base of the brain. Brain weight, 
1265 grams. There is visible atrophy of both superior frontal 
gyri and of the upper two-thirds of both central gyri. The 
extent of palpable sclerosis surpasses that of visible atrophy. 
Palpable increase of consistence is shown by the prefrontal, 
orbital (more marked on left side), frontal, central, hippo- 
campal and occipital regions. The temporal cortex is of 
normal or slightly reduced consistence. 

Section of the cerebral cortex shows everywhere preserva- 
tion of the cortical markings. The sclerosed areas show a 
diminution in depth of the cortex, which is more marked in 
the left prefrontal region. The white matter of the centrum 
semiovale of the prefrontal and occipital regions on both 
sides shows an increase of consistence. The cerebellar cortex 
also shows variations in consistence. The clivus and lobus 
cacuminis and the posterior half of the inferior surfaces of 
both cerebellar hemispheres are firmer than normal. The 
laminae of the left clivus are a trifle narrower than those of 
the right. There is visible extensive atrophy of the laminae 



TREATMENT 34I 

on both sides of a fissure in the middle of the left lobus cacu- 
minis. In the coordinate portion of the right cacumen there 
is a similar process which is less marked. The dentate nuclei 
are firm. The olives show an increase of consistence, equal 
on both sides. The left olive shows on section a crowding 
together of its folds in the middle part of the upper limb. 
Spinal cord was not remarkable. 

Summary: 

Adhesive pachymeningitis 

Chronic fibrous leptomeningitis 

Miliary pial macules 

Cerebral atrophy 

Cerebral sclerosis 

Cerebellar atrophy and sclerosis 

B ronchopneumonia 

Chronic splenitis 

Nephritis 

Aortitis 



342 TREATMENT 



It is generally recognized that DIFFUSE NEURO- 
SYPHILIS ("cerebrospinal syphilis") frequently 
is cured through antisyphilitic therapy. Example. 
Mental improvement, in one month; recovery from 
paralysis, ten months. 



Case 104. John Edwards, a man of 28 years, well de- 
developed and nourished, with general enlargement of glands 
and skin lesions, came to the hospital in a stuporous con- 
dition, with evidences of a complete hemiplegia. 

According to the wife, Edwards had had a chancre of 
the lip about a year before, for which he had been treated 
with an intravenous injection, presumably of salvarsan, and 
also presumably with mercury. The lip lesion had then 
disappeared. For a month before admission, Edwards had 
had headache and dizziness, for which he was given pills 
and drugs. There had also been difficulty with speech and 
numbness of the left arm as far up as the elbow, but this 
paresthesia had quickly disappeared. The hemiplegia was 
of only a few days' duration. After a feeling of nausea and 
vomiting, the patient had fallen with left-sided paralysis. 
Afterwards, he had shown mental peculiarities, eventually 
becoming noisy, hard to manage, and appropriate for hos- 
pital care. 

The physical examination showed a variety of increased 
reflexes, including ankle-clonus on the left side. 

The question might arise whether this case was one of 
hemorrhage or thrombosis, and the facts about the onset 
of the hemiplegia are inadequate for a decision. However, 
at so early an age, the probability of syphilis is large and the 
history of labial chancre was quite suggestive. If we may 
conclude neurosyphilis, the diagnosis of thrombosis rather 
than rupture of blood vessel is likely. The laboratory tests 
bore out the diagnosis since the W. R. of serum and fluid 
both proved positive; the gold sol reaction was syphilitic; 



TREATMENT 343 



NON-PARETIC NEUROSYPHILIS 

DIFFUSE NEUROSYPHILIS, MENINGOVASCULAR PAREN- 
CHYMATOUS, CEREBROSPINAL SYPHILIS 

CASES SYSTEMATICALLY TREATED 13 

CLINICAL RECOVERY, C.S.F. NEGATIVE 11 

UNIMPROVED I 

UNIMPROVED, BUT C.S.F. NEGATIVE I 



Massachusetts Commission on Mental Diseases, 
November, 1916 



Chart 23 



344 TREATMENT 

there were 176 cells per cmm.; there was excess albumin, 
and a positive globulin reaction. 

The outcome in such a case is dubious. If death does 
not occur soon, recovery is not impossible under treatment. 
At all events, a considerable improvement is likely. 

Edwards was given bi-weekly injections of salvarsan, 
intramuscular injections of mercury salicylate, and doses of 
potassium iodid, averaging 100 grains, three times a day. 
Under this treatment, he slowly recovered and became 
mentally clear after a few weeks. The paralysis seemed 
complete and permanent. Even after three or four months, 
there was absolutely no change in the condition, and Edwards 
was quite unable to move either arm or leg. Meanwhile, 
the spinal fluid had become practically negative to all tests. 

Treatment was somewhat optimistically continued and 
was rewarded at the end of ten months with marked im- 
provement such that the patient was able to stand on the 
paralyzed leg and move the arm to a certain degree. This 
improvement is still continuing. The spinal fluid and the 
serum have remained negative to laboratory tests. 

Note: A period of six months is commonly regarded as 
that period in which improvement in paralysis is to occur 
if there is to be any improvement. There was certainly 
not the slightest improvement in the paralysis of this case 
before eight or nine months of treatment had elapsed, and 
it took ten months to secure the marked improvement 
mentioned. 

1. What is the significance of the prodromal symptoms? 

The headache and dizziness should have been viewed 
with great gravity. They are characteristic in Men- 
ingovascular Neurosyphilis. 

Moreover in this case there had also been difficulties 
with speech and other transient symptoms which 
should have called attention far earlier to the possi- 
bility of neurosyphilis. 

2. What is the significance of the high cell count: 176 per 

cubic millimeter? Such high cell counts are frequent 
enough in diffuse neurosyphilis, but low cell counts are 
frequent also. But although the high cell count taken 
alone is of lesser significance, the fact that the high 



TREATMENT 345 

cell count In this case Is associated with a " syphilitic " 
gold sol reaction is of far greater significance for diag- 
nosis. These associated findings are characteristic of 
meningovascular neurosyphilis, 

3. What kind of recovery may be expected In successful 

examples of treatment In meningovascular cases? 
Recovery with defect. It will be noted that ten 
months elapsed before any marked improvement oc- 
curred on the paralyzed side. We could not expect a 
complete recovery from this paralysis. 

4. Was inadequacy of treatment following the chancre 

responsible for the early cerebrospinal Involvement? 
In this connection one must remember that such neural 
involvements occur occasionally even during active 
treatment (neurorecidives) . The discontinuance of 
treatment after a short period, in this case less than 
a year, is always a risk to say the least. And this is 
true even though the W. R. becomes negative, for 
trouble of a neurosyphilitic nature may occur later; 
this when both blood and spinal fluid have previously 
been found negative. The old rule of following and 
treating a syphilitic for several years despite the dis- 
appearance of symptoms is still a good rule. 



346 TREATMENT 



The results of systematic, intensive, intravenous 
salvarsan therapy in atypical neurosyphilis (cases 
not certainly paretic, tabetic or the common types 
of meningovascular neurosyphilis) may be in our 
experience as good as the results of treatment in 
common meningovascular cases: example. 



Case 105. Henri Lep^re, a machinist, 48 years of age, 
came voluntarily to the Psychopathic Hospital for a grad- 
ually failing memory and inability to work. He had had 
indigestion for four years (epigastric distress, nausea, no 
vomiting). He was still suffering from epigastric distress 
and from headaches. At times he had had difficulty in 
walking. 

Physically, Lep^re looked older than he was; he was very 
poorly developed and nourished, and seemed very weak. 
There was a slight visceroptosis. 

Neurologically, there was considerable speech defect, par- 
ticularly well marked in test phrases. The pupils were 
contracted and gave the Argyll-Robertson reaction. Neuro- 
logically there were no other signs. 

Mentally, there was a depression with worry; but it was 
a question whether these phenomena were not entirely 
natural. The special complaint was of failing memory. 

The Argyll-Robertson pupil also prima facie signifies 
neurosyphilis. Lep^re, in fact, admitted syphilitic Infection 
at 23. The gastric symptoms at once suggested tabes. The 
knee-jerks and ankle-jerks were, to be sure, preserved ; how- 
ever, this is not very unusual in tabes. The amnesia and 
aphasia naturally suggested paresis. Without resort to 
laboratory findings, accordingly, the diagnosis of tabo- 
paretlc neurosyphilis ("taboparesis") was suggested. 

The serum W, R. proved positive, but the spinal fluid W. R. 
very slightly so (yielding only moderate reaction with i cc, 
0.7 and 0.5 cc, and a negative reaction with 0.3 and o.i cc). 



TREATMENT 347 



EFFECT OF EARLY TREATMENT ON THE 
DEVELOPMENT OF NEUROSYPHILIS 

TOTAL CASES 4134 

DEVELOPED GENERAL PARESIS 198= 4.8% 

DEVELOPED TABES DORSALIS 113 = 2.7% 

DEVELOPED CEREBROSPINAL SYPHILIS. ... 132 = 32% 

443 = 10.5% 

EFFECT OF TREATMENT 

None .cour. ^^^ 

NUMBER OF CASES... 100 134 924 

DEVELOPED G.P 25=25% 31=23.1% 30=32% 

DEVELOPED TABES... II = 11% 16 = 11.9% 25=2.7% 

DEVELOPED C.S.S 3=3% 21 = 15.6% 71=7.6% 

Poorly treated Better treated 

1880-84 1895-99 

NUMBER OF CASES... 617 1139 

DEVELOPED G.P 60 = 9.7% 37=3-2% 

DEVELOPED TABES... 22 =3.5% 16= 1.4% 

DEVELOPED C.S.S 15=2.4% 28 = 2 .4% 

MATTAUSCHEK AND PILCZ 



Chart 24 



348 TREATMENT 

Globulin was moderate, and albumin was found in only 
moderate excess. There were 21 cells per cmm. in the spinal 
fluid. The gold sol reaction was that which we regard as 
typical of syphilis or tabes. If we were to rely upon the 
weakness of the fluid W. R. and the nature of the gold sol 
reaction, we should be inclined to favor the diagnosis of 
Diffuse Neurosyphilis (" cerebrospinal syphilis ") rather 
than resort to the diagnosis of paretic neurosyphilis. 

Salvarsan treatment was attended by the rapid disappear- 
ance of headaches and gastric symptoms and by a rapid 
gain in weight and feeling of well-being. Salvarsan was 
continued twice a week for two months, whereupon Lep^re 
returned to work. He has been successfully at work now for 
seven months without return of symptoms. Four months 
after beginning of treatment, the spinal fluid was examined 
and found entirely negative. Nevertheless, the serum W. R. 
has remained positive despite eight months of salvarsan treat- 
ment. 



I. What is the meaning of the titrations in the spinal fluid 
Wassermann reaction? When Plant originally applied 
the Wassermann reaction to spinal fluids, he used 0.2 of 
a cc. of spinal fluid. With this amount of fluid he found 
that cases of general paresis gave a positive reaction 
in about 100% of the cases while this positive reaction 
was only given by 40 to 60% of the cases of cerebro- 
spinal syphilis and tabes dorsalis, hence he promul- 
gated a differential point that a negative reaction in 
spinal fluid indicated that the case was not general 
paresis. Hauptmann later showed that if I cc. of spinal 
fluid were used, a positive reaction would occur in 
practically 100% of the cases of general paresis, cere- 
brospinal syphilis and tabes. Therefore, at present, 
we use the different titers of spinal fluid from which we 
draw the following conclusions: If the reaction in the 
untreated case is negative with o.i and 0.3 of a cc. and 
positive with the 0.5, 0.7 and i cc. dilutions as in the 
case of Lepere, we are probably dealing with non- 
paretic neurosyphilis. With this method of titration 
we are also better able to watch the progress of treat- 
ment as the dilutions of o.i and 0.3 cc. become negative 
first. 



TREATMENT 349 

How soon can one expect improvement after commence- 
ment of salvarsan therapy in cases of diffuse neuro- 
syphilis? The time relation of results in treatment va- 
ries with each individual case. In the case of Lep^re 
gastric symptoms that had been present for a number 
of months disappeared as if by magic after the first 
injection of salvarsan. As a rule, it is true that the 
more acute the symptoms the quicker their disap- 
pearance but this does not hold for all cases, as in this 
particular instance the long standing symptoms dis- 
appeared very rapidly. The symptoms often disappear 
very much more rapidly than the laboratoryj tests 
change. 

How can the mental symptoms (depression and failing 
memory) of which patient complained be explained? 
In the first place, as has been stated, it is doubtful if 
these are more than subjective and the result of the 
patient's feeling of discomfort and pain. However, it 
is also possible that there may be intracranial involve- 
ment of the meninges or of the brain itself. And, if 
such were the case, the improvement might be the 
result of the treatment. 



350 TREATMENT 



The Argyll-Robertson pupil should not be used as 
a basis for a necessarily bad prognosis if treat- 
ment can be given. 



Case 1 06. Frederick Stone was a business man of large 
interests. He had been in the hands of physicians for several 
years for a variety of disorders such as renal, respiratory, 
cardio-vascular, and so on. No suspicion of syphilis had 
apparently been uttered by the physicians despite the fact 
that Mr. Stone readily stated that he had had a chancre 
thirty years before, and that he had received several years' 
treatment of mercury and potassium iodid by mouth. 

It appeared that a few years ago he had begun to have 
trouble with his nose, which was cauterized and operatively 
interfered with without satisfactory results. This nasal 
condition had later been diagnosticated as gummatous, and 
had improved considerably under a mild antisyphilitic 
treatment. However, this nasal condition had been con- 
sidered and treated quite separately from the remainder of 
Mr. Stone's troubles. 

What brought him to attention was a sudden diplopia with 
ptosis. There was a paralysis of the external rectus of the 
left eye, as well as a drooping of the lid on this side. The 
left eye was much inflamed. The diplopia greatly bothered 
the patient, and there was also considerable pain in the left 
frontal region, confined chiefly to the distribution of the first 
division of the trigeminal nerve. According to the patient 
this headache was periodic. There was considerable tender- 
ness to pinprick over the area and a diminution of sensory 
discrimination of fine touch. Both the pupils failed to react 
to light. 

The remainder of the neurological symptomatic examina- 
tion was surprisingly clear of disorder, nor was there anything 
in the history suggestive of tabes. There was ozena as 
well as evidence of the operative work upon nares and throat. 
Possibly the arteries were slightly hardened; blood pressure 



TREATMENT 35 1 



PARETIC NEUROSYPHILIS 




(GENERAL PARESIS) 






Cases systematically treated 




50 




CLINICAL REMISSIONS 




34 


68% 


C.S.F. ALTERED TO NEGATIVE 


4 




8% 


C.S.F. ALTERED TO WEAKER 


16 




32% 


C.S.F. UNALTERED 


14 




28% 


CLINICALLY UNIMPROVED 




16 


32% 


C.S.F. WEAKER 


7 




14% 


C.S.F. UNALTERED 


9 




18% 


Massachusetts Commission ots 


r Mental Diseases 






November, 1916 








Chart 25 



352 TREATMENT 

was 165 systolic. There was a large trace of albumin, and 
there were numerous hyalin casts in the urine. 

Mentally, there was a degree of depression and worry 
hardly out of keeping with the general situation. Despite 
the preservation of memory, Mr. Stone failed to do rather 
simple arithmetical calculations; this was the more re- 
markable as in his business he had to handle figures a great 
deal and had been doing so until recently. There was a 
slight tremor in his writing, as well as a certain difficulty in 
enunciating test phrases. Insomnia, irritability, and a feel- 
ing of nervousness and of being tired out, completed the 
picture. 

A suggestion for diagnosis would be classically offered 
by the Argyll- Robertson pupils. Should not a patient with 
the Argyll- Robertson pupils have either tabes or paresis? 
However, in favor of tabes, besides the pupil, are to be counted 
merely the troubles with the eyes. In the direction of paresis 
we have to consider speech defect, to say nothing of less defi- 
nite symptoms such as insomnia and increased irritability. 

We are inclined to think, however, that the disease in this 
case is meningovascular. This diagnosis is suggested by the 
cranial nerve palsies and by the headache. Headache is 
much more rarely a phenomenon in the paretic type of neuro- 
syphilis than in the meningovascular type. 

In point of fact, the spinal fluid phenomena bore out the 
diagnosis of Meningovascular Neurosyphilis inasmuch as 
the globulin, albumin, cellular content, gold sol, and W. R.'s 
were all weakly positive. 

I. How far can we regard the cardiorenal defects as syphi- 
litic? Perhaps we may do so on the general principle 
of parsimony in scientific interpretation. 

The diagnostic lumbar puncture led to an extremely 
severe exacerbation of the pains on the left side of the head. 
In fact, these pains could not be held in check by the ex- 
hibition of pyramidon. Mr. Stone regarded the pain as due 
to the lumbar puncture. However, there was no improve- 
ment in the pain in the prone position, — a feature charac- 
teristic of lumbar puncture pains. Upon administration of 



TREATMENT 353 

salvarsan, this local pain rapidly disappeared. In fact, there 
was a startling improvement; the ocular palsies disappeared 
in a few weeks, although these palsies had been present 
for several months before the administration of salvarsan. 
The blood pressure was reduced ; the urine became negative. 
Perhaps the most startling feature of all (although of this we 
are not sure) was that the patient states he was accepted by 
a life insurance company although he had been twice refused 
previously. 

Note in this case the 30-year interval between infection 
and generalized neurosyphilitic involvement. Note also 
the amenability of the process despite this duration. We 
are perhaps entitled also to note that a neurological exami- 
nation careful enough to detect an Argyll-Robertson pupil 
should have been made by a number of examiners long 
before the particular crisis which we have sketched. It is 
also permissible to note that the rhinological work should not 
have been carried out independently of all other medical 
work. 



2. What are the untoward results of lumbar puncture? 
It is true that there is always a possibility of setting up 
a septic meningitis by lumbar puncture, but this is a 
very remote possibility and with any reasonable care 
it is not to be considered. Lumbar puncture also has 
a considerable danger in cases of increased intracranial 
pressure. In cases of brain tumor where the tumor is 
located in the posterior fossa, sudden death may occur 
from withdrawal of spinal fluid. This is supposed to 
be due to the medulla being pressed down into the 
foramen magnum and causing paralysis of respiration. 
Therefore lumbar puncture should never be performed 
except with the greatest caution in a case in which 
brain tumor is suspected. 

However, aside from these remote serious conse- 
quences which play very little r61e in the ordinary pro- 
cedure of lumbar puncture, certain unpleasant symp- 
toms do frequently arise. These symptoms are chiefly 
headache and nausea, but, however, may go as far as 
vomiting. These symptoms occur almost entirely in 
the cases in which there is no abnormal condition pro- 
ducing increased spinal fluid pressure. Such unpleasant 



354 TREATMENT 

symptoms may last as long as four or five days; as a 
rule, however, last only for a period of a day or two. 

3. What is the treatment of discomfort following lumbar 

puncture? It is a rule well worth observing that the 
patient after lumbar puncture should remain fiat on his 
back without a pillow for 24 hours in order to avoid any 
unpleasant symptoms. If any symptoms do occur, 
it will be almost certainly when the patient arises, and 
in nearly every instance they will be overcome if the 
patient again assumes the prone position. Raising 
the foot of the bed so as to lower the head also helps. 
Veronal or bromides may be given but as a rule are not 
very satisfactory. 

4. How permanent is the improvement obtained in the 

case of Mr. Stone likely to be? As a matter of fact, 
the patient discontinued treatment as soon as he felt 
well again, but after two months the pain returned to 
be again quickly dispelled by salvarsan. This im- 
provement must be considered as only temporary. 
Under continued treatment there may be no further 
relapse. There is, however, evidence that much dam- 
age has been done to the body by the spirochetes, 
much of which is irreparable. It is even possible that 
further disintegration might occur even while under- 
going treatment. Still treatment offers much in such 
a case and is to be highly recommended. 



TREATMENT 355 



In DIFFUSE NEUROSYPHILIS, rendering the 
spinal fluid negative by treatment may mean 
neither cure nor disappearance of sjmiptoms. 



Case 107. Greta Meyer, a widow, 51 years of age, came 
voluntarily to the hospital, seeking medical aid for a marked 
depression. She was also suffering from a right hemiplegia. 
It appeared, according to Mrs. Meyer, that she was married 
at 16, and lived with her husband until 29, whereupon she 
left him on account of his alcoholism, his abuse of her, and 
the discovery through his physician that he was suffering from 
venereal disease. She had had two healthy children and 
there never had been miscarriages or stillbirths. Six years 
after the separation, namely at 35 years of age, and 16 years 
before resort to the Psychopathic Hospital, Mrs. Meyer 
developed certain red areas on her hand, and learned at a 
hospital that these were due to syphilis. She kept up 
treatment for these lesions for a year, until she seemed per- 
fectly well. 

She had, in fact, remained perfectly well for some 14 years, 
until at 49, a small tumor had appeared on the right side of 
the forehead, near the hair line. This tumor was firm and 
not sore. Medical treatment reduced it, leaving, however, 
a depression in the bone. One day, about a month after the 
appearance of the tumor, the patient lay down for a nap, 
and upon awaking found she could only with difficulty move 
her right arm and leg. Her face was not affected; she was 
not in pain; and there was no disorder of speech. In a few 
days she got much better and she had been improving 
for some time past through the administration of further 
medicine. 

However, since the onset of the hemiplegia Mrs. Meyer 
had been very despondent. There had been ups and downs 
but she had rarely felt well. The depression was a mild one 
and in point of fact may perhaps be regarded as non-psycho- 
pathic, since at her age with her disability, there might well be 



356 



TREATMENT 



METHODS OF TREATMENT 

I. BY MOUTH. 

1. MERCURY 

2. IODIDES 

3. ARSENIC 

,11. INTRAMUSCULAR INJECTIONS 

1. MERCURY 

2. SALVARSAN, NEOSALVARSAN, OTHER ARSENIQ 

PREPARATIONS 

3. SODIUM NUCLEINATE 

4. ANTIMONY 

III. INTRAVENOUS 

1. MERCURY 

2. MERCURIALIZED SERUM 

3. SALVARSAN, NEOSALVARSAN, ARSENIC 

4. IODIDES 

IV. SPINAL INTRADURAL 

1. SALVARSANIZED SERUM (In Vivo— Swift-Ellis) 

2. SALVARSANIZED SERUM (In Vitro — Marinesco- 

Ogilvie) 

3. MERCURIALIZED SERUM (Byrnes) 

V. CEREBRAL SUBDURAL AND INTRAVENTRICULAR 

1. SALVARSANIZED SERUM (In Vivo) 

2. SALVARSANIZED SERUM (In Vitro) 

3. MERCURIALIZED SERUM 



Chart 26 



TREATMENT 357 

a degree of sadness and unhappiness concerning the future. 
Mentally, there was no other disorder of note, and in particu- 
lar no disorder of memory. 

Physically, the patient showed a right-sided hemiplegia 
with excessive right knee-jerk, but without Babinski or other 
abnormal reflex phenomena. The extra-ocular movements 
were somewhat restricted in range but there was neither 
strabismus nor nystagmus. 

The question arose whether the hemiplegia was of hemor- 
rhagic or thrombotic origin. After all, at 51 years, hemiplegia 
is rather unlikely to be of a non-syphilitic arteriosclerotic 
origin; moreover, we had a clear history of syphilis. The 
serum W. R. proved positive as well as the spinal fluid W. R. 
The finding of 77 cells per cmm., excess albumin, and positive 
globulin test, taken in connection with the entire picture 
seems to warrant a diagnosis of Cerebrospinal Syphilis^ 
If we proceed on statistical grounds, it might be regarded as 
more probable that the hemiplegia is Thrombotic in origin 
rather than hemorrhagic. It appears that syphilitic cerebral 
thrombosis rather characteristically occurs without prelimi- 
nary symptoms, despite the fact that many cases do show 
headache, dizziness, and restlessness as prodromal symptoms. 

I. What is the treatment indicated in the case of Mrs. 
Meyer? 

It would appear that little or nothing can be done 
for the hemiplegia unless the claims of Franz with 
respect to reestablishment of a degree of function in 
certain hemiplegics are substantiated. However, the 
indication of meningitic process as shown by the spinal 
fluid, suggests that the case is not a purely vascular one 
but may be regarded as meningovascular. (Possibly, 
also, we should regard the left frontal depression and 
scar as indicative of a non-parenchymatous and non- 
vascular process.) Accordingly, antisyphilitic treat- 
ment should be theoretically of some value. 

In point of fact, the patient was given injections of 
mercury salicylate, mercury by mouth, and potassium 
iodid. Her psychopathic depression under this treat- 
ment, supported by proper hygiene and rest, dimin- 
ished. However, six months later, the patient slipped 
on a wet floor and fell. Though the impact seemed 



I 






358 TREATMENT 

hardly sufficient to cause a fracture, the pelvis was 
somewhat severely fractured. Very probably there 
was a syphilitic rarefaction of the bone. Six months 
later the patient's depression was still in evidence, 
though somewhat less than upon admission. The 
blood serum remained positive but the spinal fluid had 
become entirely negative, both in respect to the W. R. 
and in respect to the other findings. 

2. How may one explain the continuance of the depression 

after the spinal fluid had become entirely negative under 
treatment? It may be that while the active process 
had been stopped, as seems probable from the negative 
spinal fluid, that a permanent destruction of brain 
tissue may account for the depression. We recognize 
this readily in instances of vascular disturbance where 
(as also in this case) the active process being stopped, a 
residual defect remains. 

3. Should treatment have been discontinued on reduction of 

the gumma? It cannot be too often emphasized that 
the disappearance of symptoms in cases of syphilis can 
not be considered as evidence of cure. The neurologist 
and psychiatrist see only too often cases of neurosy- 
philis occurring in patients who have been declared 
cured at some time previous because the symptoms then 
present had cleared up and remain in abeyance for 
years. 



TREATMENT 359 



Contrary to various warnings, arteriosclerosis by 
no means absolutely contraindicates intensive 
salvarsan therapy. 



Case 108. Victor Friedberg, 42 years of age, gave the fol- 
lowing history. He acquired syphiHs at 22 years. He had 
" adequate " medical treatment for two years with inunc- 
tions of mercury and mercury by mouth and potassium iodid. 
The only secondary symptoms were skin lesions of the legs; 
these disappeared upon treatment. Married, Friedberg has 
one child, apparently normal. There had been no miscar- 
riages or stillbirths. 

At about 34 years, there began to be shooting pains in the 
legs, occurring at first about once in three months, but later 
much more frequently. These pains were severe, lightning 
in character, lasting several days at a time, at which period 
his head would feel heavy; but there were no disturbances, 
crises, or difficulty in locomotion. 

At 36 years of age, Friedberg waked up with pain one night, 
and found he was unable to move his left leg or hand, and he 
felt his mouth drawn to the left. Upon trying to get out of 
bed, he fell to the floor. In five hours, however, he was 
entirely recovered, able to get up and walk about, and to use 
his left arm quite normally. He went to sleep, but upon 
waking up after an hour, discovered that his left side was 
again paralyzed. After two weeks in a hospital, he was able 
to walk with a crutch. The arm remained helpless for about 
a year. Both arm and leg improved slowly for two years, 
after which time his condition had remained stationary. 
For four years past, there had been no more pain, but at 42 — 
about two years before admission — the pains returned in his 
legs, back, and side. At that time he received four Injections 
of salvarsan, mercury tablets, and potassium lodld. Three 
weeks before admission to the hospital, Friedberg again 
began having headaches, very much worse than formerly. 
At first these headaches were frontal, then occipital, and there 



360 TREATMENT 

was a feeling as if something were growling inside of the head. 
There was a feeling of pressure in front on the head and at the 
base of the nose. 

Physically, Friedberg appeared somewhat older than his 
assigned age. There was a degree of general peripheral 
arteriosclerosis, but in general the physical examination was 
negative. Neurologically, there was a left hemiplegia with 
appropriate increase of the reflexes on that side, spasticity, 
Babinski reflex, and an Oppenheim ; the pupils reacted prop- 
erly ; there was no Romberg reaction. 

Mentally, Friedberg was entirely negative. 

The W. R. of the blood serum was doubtful, as was that of 
the spinal fluid. There were but two cells per cmm. and there 
was neither globulin nor excess albumin in the spinal fluid. 

The differential diagnosis might lie between cerebral 
hemorrhage and syphilitic thrombosis. Thrombosis is much 
more common as a result of syphilis than is hemorrhage. 
The occurrence of the thrombosis during sleep without pre- 
monitory symptoms is also characteristic in syphilis. Pos- 
sibly there was a low-grade spinal meningitis at the bottom of 
the lancinating pains. Whether the headache is an arterio- 
sclerotic effect or due to a meningitis not shown in the 
cerebrospinal fluid is doubtful. However, the absence of in- 
flammatory products in the cerebrospinal fluid rather indicates 
that the headache is of arteriosclerotic origin. Autopsies, 
however, warn us that we may have a localized meningitis 
in various parts of the cranial cavity without the determina- 
tion of any inflammatory products in the spinal fluid. 

I. How shall we explain the doubtful (slightly positive) 
W. R. in the spinal fluid if the case is one of Vascular 
Brain Syphilis? The finding is not unusual in these 
cases. The W. R. producing body is recognized to be 
of a separate nature from the globulin and albumin 
bodies, and is probably also separate from the gold sol 
reaction producing bodies. 

Treatment: The theory of treatment is that any spi- 
rochetes that may be still active in the body should be de- 
stroyed. Accordingly, although salvarsan can certainly have 



TREATMENT 361 

no effect in reproducing nerve tissue, it nevertheless seems 
indicated. It is frequently stated, however, that salvarsan 
is dangerous in cases of this group. We have not found this 
statement correct. In this case, there was a symptomatic 
improvement, as far as pain and discomfort went, under 
salvarsan and iodids. 

2. What precautions should be taken in intensive salvarsan 

treatment of syphilitic arteriosclerosis? Treatment 
should be begun with very small doses of salvarsan, 
that is, about o.i of a gram and then the amount slowly 
increased. The injection should be given slowly so as 
not to put too great a load upon the cardiovascular 
system. 

3. What r61e does the mental attitude of the patient play in 

a case like that of Friedberg? It was quite evident 
that Friedberg was neurotic and that he had a syphil- 
ophobia. Consequently some of the symptomatic im- 
provement may have been more results of assurances 
offered by the physician and knowledge that he was 
being treated, than results of salvarsan. In some cases 
mental anguish suffered by the patient is of more 
importance than the actual symptoms of the disease 
and this point must be always borne in mind in handling 
syphilitic patients. 



362 TREATMENT 



Symptoms of intracranial pressure cured by anti- 
syphilitic treatment. 



Case 109. Mrs. Annie Rivers, a housewife 36 years of age, 
sought advice and treatment for severe convulsions which 
she had had during a period of several weeks. She left the 
hospital before being properly examined, and had several 
more convulsions, after which she was brought back in a state 
of marked confusion. The confusion shortly disappeared 
almost completely, and a good history was obtained. 

It appears that the patient led a normal life and had had 
six children, the last of whom was born about four months 
before her coming to the hospital. The first symptoms 
appeared about a month after the birth of the child, when, 
one afternoon, Mrs. Rivers suddenly fell unconscious while 
ironing. She remained unconscious for nearly three hours. 
During this attack there were no convulsive movements or 
tongue biting; and after the spell, she felt neither lame nor 
sore, but merely tired. This was Mrs. Rivers' statement; 
but her daughter stated that the patient really did have con- 
vulsive movements. A week later came a second convul- 
sion, followed by daze and stupor. This second attack 
lasted two hours. 

About a week before entrance, the patient had remained 
in bed on account of dull grinding pain in the left side of the 
head, below the ear, and upon this day the patient vomited 
twice. In addition to the dull grinding pain, there were 
pains referred to the ear itself and to the left side of the head, 
especially over the left eye; there were no pains on the right 
side of the head. The next day the patient was better, but 
the day thereafter again remained in bed. The only other 
symptoms were cold feelings at times and bright spots in the 
field of vision. 

No mental symptoms were observed in Mrs. Rivers except 
a bit of depression after her hasty retreat from the hospital 
the first time. Upon her second admission, however, after 



TREATMENT 363 



UNTOWARD SYMPTOMS OF THERAPEUTIC 
AGENTS 

A. SALVARSAN 

CYANOSIS MALAISE 

RAPID PULSE 

PERSPIRATION 

RESPIRATORY DIFFICULTIES 

FEVER 

NAUSEA, VOMITING, DIARRHOEA 

DERMATOSES 

EDEMA 

KIDNEY IRRITATION 

LIVER IRRITATION 

INTENSIFICATION OF SYMPTOMS 

COLLAPSE 

B. MERCURY 
SALIVATION 

FETID BREATH 

EXCESS FLOW OF SALIVA 

TENDERNESS OF TEETH — LOOSENING AND FALL- 
ING OUT 

SPONGY GUMS — EROSION 

METALLIC TASTE 

NECROSIS OF BONES OF JAW 

SORENESS OF PAROTIC AND MAXILLARY GLANDS 

SWELLING AND EROSION OF TONGUE AND MUCOUS 
MEMBRANES 
CASTRO-INTESTINAL SYMPTOMS 
ANEMIA 

PAIN IN JOINTS 
NEPHRITIS 

C. IODINE 
SKIN LESIONS 
METALLIC TASTE 
SALIVATION 
CORYZA 

URTICARIA (EVEN TO GRADE OF ANGIONEUROTIC EDEMA) 
PAINS 

CONSTIPATION 
INVOLVEMENT OF JOINTS 
FEVER 

SOFTENING AND BLEEDING OF GUMS 
EROSION OF MUCOUS MEMBRANES 
GASTRO-INTESTINAL SYMPTOMS 
ANOREXIA 
WEAKNESS 



Chart 27 



364 TREATMENT 

a week or ten days* residence, apathy developed together 
with considerable amnesia for the same facts she had quite 
readily remembered a few days previously. Along with the 
apathy and amnesia developed considerable headache; and 
there were attacks of vomiting. 

On the physical side, it is interesting to note that the oph- 
thalmoscopic examination upon Mrs. Rivers' first admission 
to the hospital was entirely negative, whereas a week later, 
pronounced difficulty with vision appeared so that in a few 
days she was able to make out only very large type. The 
fundi now showed hazy and indistinct disc outlines, with 
small yellowish areas of fatty degeneration above the disc, 
reduction of arterial calibre, and dilated and somewhat tor- 
tuous veins (no projection of papillae), so that the ophthal- 
mological diagnosis was chronic neuritis. 

The physical examination otherwise was mostly negative. 
The skin presented irregular areas covered with silvery scales 
over the arms and chest, back, abdomen, and legs (the patient 
had had psoriasis several years before). Both pupils reacted 
to light and distance, though the right was slightly larger 
than the left and somewhat irregular. There was a slight 
tremor of the tongue and extended fingers. The reflexes 
were active, especially the knee-jerks; no abdominal reflexes 
could be obtained. The serum W. R. was positive, but the 
spinal fluid W. R. was negative. The spinal fluid showed 
but 3 cells per cmm., but there was a positive globulin test 
and an excess of albumin. 

Diagnosis: After the symptoms had fully developed, it 
became clear from the optic neuritis, headaches, and vomiting 
that a condition of intracranial pressure existed. In view 
of the positive serum W. R., it is natural to conceive that the 
agent producing the Intracranial pressure was a gumma. 

It is, of course, possible that a marked degree of meningitis 
might be so localized as to produce the same symptoms. The 
diagnostician would crave a pleocytosis of the spinal fluid 
if a diagnosis of meningitis is to be made ; and there was no 
such pleocytosis. On the whole, we do not feel that it is 
possible to make a diagnosis either of Meningitis or of 
Gumma. 



TREATMENT 365 

Treatment: Treatment, however, caused a disappearance 
of all symptoms. The treatment consisted of but one injec- 
tion of 0.3 gram of salvarsan, followed by a few injections of 
mercury; whereupon Mrs. Rivers became much brighter, 
recovered her vision, lost her headaches, ceased to have con- 
vulsions or vomiting spells. 

I. Is salvarsan contraindicated in cases with involvement of 
the optic or auditory nerves? Such a contraindication 
exists according to prevailing opinion. In this partic- 
ular case, a hemorrhagic retinitis occurred after the 
injection of salvarsan, but this retinitis disappeared 
along with the other symptoms. On the whole we 
believe that in many cases of optic or auditory nerve 
involvement salvarsan should be used. However, one 
should never lose sight of the possibility of untoward 
results and should advise such treatment only when 
other treatment seems inefficient. 



366 TREATMENT 



TABETIC NEUROSYPHILIS (''tabes dorsalis ") 
may show very marked improvement as a result 
of intraspinous therapy. 



Case no. Mr. McKenzie* was a retired merchant of 42 
years whose complaint was that he tired very easily, could not 
make his legs go where he wished, was unsteady and felt a 
numbness in his legs. These symptoms had been in prog- 
ress for a few months only when the examination was made. 
This disclosed Argyll-Robertson pupils, absent knee-jerks 
and ankle-jerks, Romberg sign, unsteady gait, moderate 
ataxia and dysmetria. The W. R. was negative in the 
blood serum but positive in the spinal fluid with 0.2 cc, and 
there were 107 cells per cmm. With the symptoms and signs 
it was therefore easy to make the diagnosis of Tabetic 
Neurosyphilis {" tabes dorsalis "). 

The patient was given five intraspinous injections of mer- 
curic chloride in blood serum (mercurialized serum) according 
to the method of Byrnes. The dose was o.ooi gm. of mercury. 
Two weeks after the first injection the cell count was 58 cells 
per cmm., the Wassermann was positive only with 0.4 cc. 
After the fourth injection there were but 18 cells and the 
Wassermann reaction was negative even with i| cc. of spinal 
fluids. The symptoms had improved to such a degree that the 
patient had no complaint whatsoever and considered himself 
cured. 

I. What are the unpleasant results of intraspinous therapy? 
Frequently there is an exacerbation of symptoms and 
pain may be quite severe after intraspinous injections. 
This, however, lasts only a short period, that is, as a 
rule less than 24 hours. There may be other symp- 
toms of cord irritation as retention of urine or lack of 
sphincter control. A rise of temperature is not unusual. 

* (This case was furnished by Dr. D. A. Haller from the 
Peter Bent Brigham Hospital series.) 



TREATMENT 367 



Treatment may alter the W. R. to negative in 
blood and spinal fluid in TABES DORSALIS. 



Case III. Ivan Rokicki was a baker, 43 years of age, who 
came complaining of exceedingly severe attacks of abdominal 
pain with vomiting. He described these attacks as haying 
occurred periodically for a number of years, lasting sometimes 
as long as a week, during which time Rokicki could not eat or 
get relief short of large doses of morphine. 

Upon his arrival, Rokicki was seen in one of his attacks; 
he was curled up with excruciating pain, and the abdomen 
was rigid, though it was impossible to produce additional 
pain by external pressure. There was spasmodic vomiting, 
frequently followed by slight relief from the pain, which 
however shortly recurred and caused the patient to cry out in 
his suffering. The condition was controlled by opiates but 
lasted a full week. The leucocytes remained normal and 
there was no rise of temperature. The attack ceased spon- 
taneously. 

Save for the pain, Rokicki 's mental examination proved 
entirely negative. Physically, Rokicki was fairly well devel- 
oped and nourished. His pupils were slightly irregular: the 
left markedly larger than the right; both pupils failed to 
react to light, and the left pupil also failed to react In ac- 
commodation. There were no other reflex disorders evident 
to systematic examination, nor was there sensory distur- 
bance or speech defect. The heart seemed somewhat en- 
larged but there were no murmurs; blood pressure: systolic 
150; dia'stollc no. 

The correct symptomatic diagnosis In Rokickl's case proved 
to be gastric crises, and this diagnosis must perforce be the 
first to entertain In view of the chronlclty, the periodicity, 
the non-relation to diet, and the spontaneous cessation of 
the seizures. The observation of Argyll-Robertson pupils 
was naturally held to substantiate the diagnosis of Tabes 

DORSALIS. 



368 TREATMENT 

The possibility of abdominal inflarriTnation could be shortly 
dismissed on account of the absence of tenderness (the rigid- 
ity in this case was not accompanied by tenderness), fever, 
and other characteristic signs. There was no diarrhoea, such 
as is found in lead colic, and there was no other sign of 
plumbism. Jaundice was absent and there was no special 
radiation of pain from the abdomen. One had to think of 
gastric ulcer and hyperchlorhydia, and possibly malaria or 
gastroenteritis. 

The pupillary reactions pointed to a syphilitic condition 
despite the fact that the lack of reaction to accommodation 
(over and above the Argyll- Robertson phenomenon) in the 
right pupil is not entirely typical. Accordingly, although 
there was no areflexia, Romberg sign, or ataxia, resort was 
had to the W. R. This however proved negative, in blood 
and spinal fluid ; nor was there any globulin or excess albu- 
min; there were 5 cells to the cmm., in the spinal fluid. 

We are left, accordingly, with characteristic gastric crises; 
Argyll-Robertson pupils, slightly irregular; and a somewhat 
enlarged heart. 

Upon investigation, it appeared, however, that a year 
before the attack above described, the patient had been 
examined and both blood and spinal fluid found positive to 
the W. R. At that time, treatment, consisting of intravenous 
injections of salvarsan and intraspinous injections of salvarsan- 
ized serum (Swift-Ellis), had been instituted. Whereupon 
the laboratory tests had become negative, as above stated, 
and there had been no alleviation of the symptoms. 

1. How can Rokicki's normal deep leg reflexes be explained? 

The abolition of the deep reflexes is of course due to 
lesions properly localized. It is probable that this 
particular case of tabes dorsalis is more truly " dorsal " 
than most cases ; for most cases exhibit lesions involving 
regions lower than the dorsal. Both in these dorsal 
cases and in certain rare cases of cervical tabes, the 
deep leg reflexes are preserved. (See cases Green (30) 
and Halleck (31).) 

2. What is the mechanism by which a characteristic gastric 

crisis is produced? The mechanism is unknown. Some 
endeavors have been made to meet gastric crises by 



TREATMENT 369 

surgery of the posterior roots, on the assumption that 
the irritation causing the pain was located either in 
the posterior gangUon or in the passage of the nerve 
through the meninges. In only a few instances, how- 
ever, has the result been what was desired. In many 
instances the gastric crises and pain continued uninter- 
rupted and in addition came discomfort due to the lack 
of sensation in the part supplied by the severed nerve. 
At present this treatment is seldom carried out. 
Should antisyphilitic treatment be continued in such a 
case? As far as our present knowledge of syphilis goes 
one would hesitate to suggest further antisyphilitic 
treatment, feeling that the active process had been en- 
tirely stopped as suggested by the absence of any posi- 
tive findings either in the blood serum or in the spinal 
fluid. We should perhaps conclude that there was no 
more activity in this case and that the crises were due to 
the changes that had already taken place in the nerve 
^ tissue and which could no longer be changed. 



I 



370 TREATMENT 



The literature is in doubt concerning (in fact is 
preponderantly against) the success of treatment 
in PARETIC NEUROSYPHILIS (''general par- 
esis "). Our experience has yielded a number of 
apparently successful results through systematic 
intensive intravenous salvarsan therapy. Example. 



Case 112. Albert Forest had always been a successful 
salesman, but in the middle of March, in his 46th year, he 
was arrested for grabbing a purse from a woman in front of 
a theatre and running down the street with it. In court, 
Forest acted strangely and he was sent to the Psychopathic 
Hospital for observation. Upon investigation, it appeared 
that his wife thought he had been showing mental changes 
for about a year. For example, he would embrace his wife 
on a street car, or refuse to pay her fare. He once attempted 
to hit his son on the head with a red-hot poker. Now and 
then he would become sleepy and stupid. He looked rather 
older than his age and had a coarse tremor of the hands. 
Otherwise, no change could be detected In the physical 
examination, either neurologlcally or otherwise. As for the 
manual tremor, Forest's wife gave a history of considerable 
alcoholic Indulgence on his part. 

For several days, nothing abnormal could be detected In 
the man ; and In particular, his memory for both remote and 
recent events was very good and his knowledge of current 
events was good. Simple arithmetic was easy to him. 

One evening his temperature was found to be 104° F. and 
no cause could be discerned for this. The next morning. 
Forest was discovered In a stupor, with a complete right 
hemiplegia. The BablnskI reflex, the Oppenheim reflex, and 
ankle clonus had appeared on the right side, and the right 
arm was spastic. 

However, all symptoms of this paralysis had disappeared 
by four o'clock In the afternoon, and the paralytic phenomena 
were replaced with violence. The patient fought with the 



TREATMENT 37 1 

attendants and for some time remained extremely difficult to 
manage, being confused and subject to outbreaks of violence 
with destruction of furniture and other property about the 
ward. 

Diagnosis. At first we were naturally inclined to dismiss 
the case with a diagnosis of alcoholism. The transient 
hemiplegia at once raised a considerable question of brain 
syphilis or of brain tumor. 

The W. R. of the serum was doubtful. The spinal fluid 
yielded, besides marked excess of albumin and much globulin, 
also a " paretic " gold sol reaction and 75 cells per cmm. 
The W. R. was positive. 

Treatment. The patient was given injections of salvar- 
san, 0.6 gram, twice a week, with potassium iodid. After 
a few weeks Improvement followed, and after several months 
all the laboratory tests became negative, the patient was 
apparently perfectly normal mentally and was discharged 
from the hospital, and has remained well for 18 months 
without further treatment. The serum W. R. has con- 
tinued to be negative. 

1. What is the significance of the so-called " doubtful " 

W. R.? Where there is not a complete uniformity the 
results of the strong and weak antigens (see appendix 
on technique of Wassermann reaction) the result is 
reported as doubtful. In the majority of instances 
repetitions will give a strong positive reaction. 

2. Is the case of Forest to be regarded as one of general 

paresis? Sometimes such cases are termed in the 
literature syphilitic pseudoparesis (see case Burkhardt 
(58)). The differential diagnosis of this group is 
entirely therapeutic. There are, unhappily, no labora- 
tory tests which will suffice in the present stage of 
knowledge to differentiate a case of so-called pseudo- 
paresis from general paresis. We are inclined to term 

the case one of General Paresis, with recovery, or, 

at all events, with remission. 



372 



TREATMENT 



The literature is in doubt concerning (in fact is 
preponderantly against) the success of treatment 
in PARETIC NEUROSYPHILIS ('* general par- 
esis "). Our experience has yielded a number of 
apparently successful results through systematic 
intensive intravenous salvarsan therapy. Example. 



Case 113. We present the case of Gussie Silverman, a 
housewife, 35 years of age, among other reasons, for its social 
interest. The case is, on the whole, sufficiently typical of 
General Paresis. Physically, for example, the pupils failed 
to react to light and accommodation and were unequal, the 
right being larger than the left. The knee-jerks were sluggish 
though equal. The ankle-jerks could not be obtained. The 
abdominal reflexes were not obtained. Otherwise, there was 
no reflex disorder. 

From the laboratory point of view, the W. R. was positive 
in the blood and in the spinal fluid. There were 80 cells per 
cmm. and there were an appropriate globulin and albumin 
reactions. Mrs. Silverman was rather poorly nourished and 
had a slight edema of the ankles. 

Mentally, she was found on admission to be markedly 
depressed. It appeared that during a recent pregnancy, 
terminated by the birth of a 7-months child, she had fainted 
several times a day, that since the confinement she had been 
very nervous, that she had been asking her husband not to 
send her away, that she had refused to leave the house, that 
she had become excited even to the point of Injuring herself, 
especially at night, and that she would go so far as to scratch 
her husband, shortly afterward being very sorry for her per- 
formances. Before this last pregnancy there had been four 
others and the resulting children were all apparently In good 
health. Except for the fainting spells during the pregnancy, 
it would not appear that the story just told is at all charac- 
teristic of paresis. 



TREATMENT 373 

However, In the hospital Mrs. Silverman could hardly be 
got to answer questions, continually saying, " You know what 
it is; I don't have to tell you." She claimed so marked a 
degree of confusion as not to know where she was and what 
she was doing. She would beg despondently that something 
be done for her, and iterate and re-iterate these claims. 
There appeared to be a marked degree of amnesia. Some 
one, she felt, had controlled her thoughts and made her do 
things she did not want to do and say things she did not want 
to say, things she did not know she was about to say. She 
said, " I feel like jumping around. I couldn't believe myself 
as if I am me. Some one is making me jump around. I used 
to hear him talking. I don't know who it is. I used to keep 
my eyes open and I couldn't move. I feel only I would like 
to talk, and talk, and talk, and talk all the time. It seems to 
me that some one talks in me. I couldn't sleep for five min- 
utes. My God, I wish I could sleep! I used to feel something 
in my heart. I used to faint. It seems to me I used to see 
a funny thing. What it was I can't tell. It used to talk to 
me, make me get out of bed, throw me about, make me do 
things. O, I don't know what it was." 

I These not entirely characteristic mental symptoms, to- 
gether with the suggestive physical signs and the laboratory 
examination, caused treatment to be instituted ; under which 
treatment (intravenous Injections of salvarsan) she improved 
rapidly. Mental symptoms disappeared under the ad- 
ministration of 12 injections of salvarsan within two months. 
Moreover, the spinal fluid became entirely negative. Two 
and a half years have now elapsed since her discharge and she 
has shown no return of symptoms. The serum W. R. has 
always remained negative although there has been no treat- 
ment since leaving the hospital. There has, however, been 
no change In the reflexes, which remain as on admission. The 
7-months baby has continued to be perfectly healthy. Its 
W. R. Is negative, as are the W. R.'s of the husband and the 
other three children. It must seem surprising that a healthy 
child could have been born from a mother with generalized 
syphilis as in this case. However, perhaps there are more 
Instances than we imagine like the case of baby Silverman. 



374 TREATMENT 

1. May a patient be considered permanently cured although 

there has been no recurrence of symptoms for 2| years 
and although the Wassermann has remained negative? 
One would hesitate to give a definite statement that the 
patient was cured until more time had elapsed. It is 
quite possible that spirochetes may be lurking In some 
portion of the body without causing the production of 
symptoms or Wassermann bodies and yet ready to 
break out at any time. This hypothesis has added 
weight from the recent work of Warthin already quoted. 
We advise examination of this patient at Intervals of 
not longer than six months for a good many years. 

2. Should the course under treatment cause us to change 

the diagnosis? It has often been stated that a differ- 
ential point between cerebrospinal syphilis and general 
paresis Is the reaction to treatment, that is, that a case 
which recovers could not be general paresis. Head 
and Fearnsides state that if six months after beginning 
of treatment the spinal fluid has become negative, the 
case should be considered as one of cerebrospinal 
syphilis and not general paresis. We do not feel ready 
to concur in this view as we know of no similar logic in 
medicine. We have many cases in which a spinal 
fluid has remained positive for six months and later 
become negative, so that where the symptoms shown 
are those of paretic neurosyphilis, we are inclined to con- 
sider the case such until such time as more definite 
evidence checked by post mortem examination causes 
us to change this point of view. 

3. Do the reflexes change under treatment? The signs of 

spasticity often do disappear under treatment and also 
when there is no treatment. A few instances have 
been reported in the literature where Argyll- Robertson 
pupils are said to have altered to normal. It has never 
been our good fortune to see such a change nor have 
we seen an absent knee-jerk become normal, as has 
also been reported, except where It Is the result of 
pyramidal tract disease superimposed upon the pos- 
terior column sclerosis causing a return of reflex. This, 
of course, Is not to be considered as a return of the 
normal. (See Case I.) 



TREATMENT 375 



Some RESULTS of systematic intravenous salvar- 
san therapy are PARTIAL (e.g., clinical recovery 
and persistence of positive laboratory tests). 



Case 114. Walter Henry was an undertaker in a small 
town. He was married and the father of two healthy children. 
In May, 1914, he began to lose his appetite. He felt restless 
and seemed to be losing his grip, and in August he repaired 
to a sanatorium, where he remained for two months. Shortly 
after leaving the sanatorium, he fainted one day, while dig- 
ging a grave, during a spell of great heat. Since that time 
there had been numerous " weak spells," with headaches and 
general debility, insomnia, and loss of weight. 

In February, 1916, Mr. Henry came to the hospital for 
advice, but the trip from a distant part of the state was ap- 
parently such a strain for him that shortly after admission 
he collapsed. There were no convulsive movements in this 
collapse, but the patient was confused and his breathing was 
rapid and stertorous. The semi-stupor lasted for about 48 
hours. Upon recovery from the stupor, Henry was found 
entirely disoriented, much confused, and laboring under the 
belief that he was digging a grave. After a time he again 
fell into a stupor and his temperature rose to 103° F. 

The emaciation of this man was striking and unusual, 
but systematic physical examination showed no special dis- 
ease. Neurologically, there were marked tremors, and there 
were purposeless movements of the arms. There was a 
marked speech defect. The pupils were dilated, regular, and 
equal, and reacted, though slightly, to light. Nothing ab- 
normal was noted upon systematic examination of the reflexes. 

The W. R. was strongly positive in the blood and in the 
spinal fluid; the gold sol reaction was typically "paretic"; 
there were 16 cells per cmm., globulin was present, and albumin 
was greatly increased. 

The diagnosis General Paresis was accordingly made, and 
treatment instituted. Intravenous injections of arsenobenzol, 



376 TREATMENT 

at first, and later of diarsenol, were given, as a rule twice a 
week (usual dose, 0.6 of a gram). Mercurial injections and 
potassium iodid were also given. This treatment was con- 
tinued as the patient began to improve. The improvement 
was of such a degree that at the end of four months, Mr. 
Henry returned to his home and his work. He had had 30 
intravenous injections of salvarsan substitutes. Despite the 
treatment and the clinical improvement, the laboratory tests 
remained essentially unchanged. The W. R.'s of the blood 
and spinal fluid remained strongly positive, as well as also the 
globulin and albumin; the gold sol reaction was still "paretic"; 
the cells stood at one per cmm. The patient has continued 
antisyphilitic treatment since leaving the hospital, and has 
remained apparently well, with good insight into his condition. 

1. What is the significance of a temperature of 103° in a 

paretic without signs of infection and a normal leuco- 
cyte count? Temperatures of this type are not infre- 
quent in the course of general paresis. They are usually 
spoken of as " paretic temperatures." Their meaning 
is not understood, but they are often stated to be due to 
a disturbance of the heat-regulating mechanism. Such 
temperatures ma}^ remain elevated for a considerable 
period of time, but the elevation may be very transi- 
tory. At times they vary, like septic temperatures. 

2. What can be argued from the fact that the cell count 

became normal? If thorough antisyphilitic treatment 
is vigorously given, it will be found that in the vast 
majority of cases of neurosyphilis the cell count will 
return to normal. It matters not whether the treat- 
ment be intravenous or subdural. It is very difficult, 
however, to obtain this result in general paresis by the 
use of mercury alone. It cannot, however, be urged 
that this finding has any great prognostic significance 
as it occurs in the cases which do poorly as well as in 
those which recover symptomatically. 

3. Is it safe to give large doses of salvarsan to a patient in 

a stupor? It is not a good plan to give a large dose to 
such a patient on account of the danger of sudden 
death. This is probably due as much to the strain 
put on the heart as it is to any effect on the nervous 
system, or specific arsenic efl^ect. In this particular 
instance, a dose of 0.15 gm. was the initial Injection 
and this was increased five centigrams per injection. 



TREATMENT 377 



IMPROVEMENT IN PARETIC NEUROSYPH- 
ILIS ('* general paresis ") may become evident 
only after several months of intensive treatment. 



Case 115. Henry Ryan was a shipping clerk, 54 years of 
age, who was brought to the hospital following a convul- 
sion. For a few months preceding this period, Mr. Ryan 
had been failing in his abilities. He had been very forgetful, 
showed no energy, and had become very irritable. He also 
complained of insomnia and of feeling nervous. 

On admission to the hospital, the most striking feature in 
the mental situation was that he claimed that he had not 
slept a wink for three months, and each day he would solemnly 
affirm that he had not slept at all the preceding night, although 
the records might show that he had slept eight hours. Argu- 
ment was of no avail against this conviction. In addition, 
his memory was very poor; he showed little knowledge of 
current events, and had no ability with arithmetical problems. 

Neurologically viewed, the points of chief significance were 
contracted immobile pupils and a speech defect, especially 
noticeable on the repetition of test phrases. The whole 
picture was suggestive of general paresis, and this diagnosis 
was confirmed by the laboratory findings. It was found that 
the W. R. was positive in the blood and spinal fluid, that 
there was a pleocytosis, positive globulin reaction, excess of 
albumin, and a " paretic " gold sol reaction. Consequently, 
the diagnosis of General Paresis seemed justified, although 
the patient denied any knowledge of a syphilitic infection. 

Treatment in this case consisted of intravenous Injections 
of salvarsan, diarsenol, or arsenobenzol, whichever drug was 
most easily obtainable, given twice a week in doses of 0.6 
gram each. In addition, he was given occasional injections 
of mercury salicylate as well as potassium lodid by mouth. 
Once or twice a week, 40 to 60 cc. of spinal fluid were with- 
drawn. Under this treatment for a period of three months, 
the patient showed no improvement whatsoever, either in 



378 TREATMENT 

his mental condition or in the laboratory findings. However, 
treatment was faithfully persevered in, and shortly after the 
three months, improvement began to be noticed. At first, 
the patient began to admit that possibly he may have slept 
a few winks some time during the previous six months, for 
he said he realized it was not possible for a man to live without 
sleep for that period. Then he began to admit that he might 
have slept a few hours during the night, and later that he 
was sleeping pretty fairly. His memory also showed im- 
provement. His general attitude showed alertness, and he 
began to interest himself in his surroundings and in the events 
of the world, and finally he gained complete insight into his 
condition. 

In the meantime, that is after three months of treatment, 
the laboratory findings began to grow weaker. The gold sol 
reaction was the first to decrease in strength, and after four 
months of treatment, it vacillated between negative and a 
mildly positive '' syphilitic " reaction. Then the globulin 
and albumin became less in amount, and the W. R. began 
dropping off in the o.i and 0.3 cc. dilutions. As is usually 
true in those cases of neurosyphilis that receive adequate 
treatment, the cell count early dropped to normal. The 
W. R. in the blood serum, however, remained positive. 

As the patient's condition seemed so much better, he was 
allowed to leave the hospital at the end of five months. He 
took things easily for the following seven months, and then, 
after being out of employment for the period of a year, as his 
health continued good, he decided to return to work. Before 
doing so, he entered the hospital again for a lumbar puncture. 
At this time, it was found that the cell count was normal, 
there was a very faint trace of globulin, possibly a slight in- 
crease above normal albumin content, and a very mild gold 
reaction. The W. R. in the spinal fluid was negative includ- 
ing the i.o cc. dilution; the blood serum remained positive. 

The patient then returned to his old position and has done 
satisfactorily for the past six months. During this entire 
time, he has been coming to the hospital for treatment: 
during the major portion of the time, about once in two weeks; 
of late, once in four weeks. 



TREATMENT 379 

The significant point in this case is that improvement did 
not show itself until after more than three months of intensive 
treatment, and then the improvement was synchronous with 
a weakening of the spinal fluid tests. 

It is further significant that his mental and physical condi- 
tion was good before the tests had reached anything like 
normal; and that under treatment, these tests continued 
to grow weaker and weaker, until at the end of a year, they 
were practically negative. 

The case further illustrates the enormous number of in- 
jections of salvarsan preparations that may be given to a 
patient without causing any appreciable damage to the gen- 
eral health or to the kidney function. Mr. Ryan has had 
more than 60 injections. 

1 . How soon after treatment is instituted does improvement 

usually occur in paretic neurosyphilis? In our ex- 
perience improvement usually shows itself in from two 
or three months of treatment. Occasionally the im- 
provement may be very marked shortly after treatment 
is commenced, that is, after three or four injections of 
salvarsan. This is not, however, the rule and as in 
the case of Ryan, it may be only after more than three 
months that improvement is seen. This means that 
in the treatment of these cases patience must be exer- 
cised and much work done. 

2. What is the point of withdrawing large amounts of spinal 

fluid as in the case of Henry Ryan? It has been stated 
that the withdrawal of 40 or more cc, of spinal fluid 
while the patient is under treatment has the effect of 
reducing the intraspinous and intracranial pressure and 
thereby allowing the drug to diffuse into the nervous 
tissue better than it would do under ordinary condi- 
tions. How much truth there is in this contention it is 
difficult to say and there is as yet no experimental evi- 
dence to confirm this contention. As a matter of fact, 
the spinal fluid in cases of paresis is usually under 
increased pressure and it is at least plausible to conceive 
that a reduction of this pressure may give some symp- 
tomatic relief. 



38o TREATMENT 



Evidence of the activity of syphilis outside the 
central nervous system may be seen in cases of 
neurosyphilis despite intensive treatment. 



Case ii6. William Rossetti was a speculator, 43 years of 
age, when he was brought to the Psychopathic Hospital on 
account of an^outbreak in which he smashed a showcase at 
the store where his sweetheart was employed; he caused so 
much commotion that he was arrested. 

On admission, he was very excited, talking loudly and at 
length. For some days it was very difficult to manage him, 
he was so active. At any moment, he would insist upon 
undressing and taking physical culture exercises. He was 
very euphoric and expansive, and had no insight into his 
condition. 

Physically, he was a powerfully-built man and in very good 
physical condition except for an iritis and moderate thickening 
of the peripheral arteries. The neurological signs of import- 
ance were Argyll-Robertson pupils, and absent knee-jerks 
and ankle- jerks. With these findings in mind, a tentative 
diagnosis of General Paresis was made, and this was sub- 
stantiated by the laboratory tests, which gave positive W. 
R.'s in blood and spinal fluid, globulin, excessive albumin, 
slight pleocytosis, and a " paretic " gold sol reaction. 

When the patient's mental condition was somewhat better, 
he gave a history of syphilitic infection 15 years before, for 
which he had had almost continuous treatment. As a matter 
of fact, treatment had been pretty strenuous because he had 
recurring skin lesions and Iritis. It was practically impos- 
sible to get the skin lesions to heal with mercury, and it was 
not until salvarsan was introduced that a good result was 
obtained in this respect. After one or two injections of this 
drug, the skin lesion disappeared and has never returned. 
However, at least once a year, he has had attacks of iritis, 
and for this reason was still being treated for syphilis at the 
outbreak of his psychosis. 



TREATMENT 38 1 

He was at once placed on more strenuous antisyphilitic 
treatment in the form of diarsenol, semi-weekly, aided by 
mercury injections. After a few months of this treatment, 
his mental condition improved so much that he seemed to be 
entirely normal. Treatment was continued, however, with- 
out any abatement, and it was of great interest to note at the 
end of five months of such treatment that, although mentally 
he seemed entirely well, he had an attack of iritis, which was 
considered as a sign of active syphilis. This would appear 
to indicate the great difficulty of getting results in certain 
cases of syphilis with any drugs at our command at present, 
as in the iritis we are dealing with a condition which as a 
rule reacts fairly readily to antisyphilitic remedies. 

1. Are there different strains of spirochetes showing various 

degrees of malignancy? This question has been dis- 
cussed at length in the literature but there is no satis- 
factory answer at the present time. We must always 
consider the reaction of the organism and the host ; and 
it is true in syphilis, as in every other disease, that in 
some individuals it is more difficult to get any therapeu- 
tic results than in others. 

2. Was the failure to obtain results by long years of treat- 

ment due to "drug fastness" of the spirochetes? It has 
been held that the organism of syphilis will develop an 
immunity after a time to mercury and arsenic prepara- 
tions. This led Fournier to recommend intermittent 
treatment as more efficient than continuous treatment. 
Noguchi has shown that in test-tube experiments, the 
spirochetes develop a tolerance to increasing doses of 
arsenic. It must be emphasized, however, that this find- 
ing has not been established for the conditions in vivo. 
Another explanation of the failure of treatment in certain 
instances has been offered by McDonagh, who describes 
a life cycle of the organism of syphilis under the name of 
cytorrhyctes luis, of which he believes the spirochete to be 
merely one form, the other forms not being affected by 
arsenic or mercury. 



[ 



382 



TREATMENT 



Some results of systematic intravenous salvarsan 
therapy in PARETIC NEUROSYPHILIS (" gen- 
eral paresis ") are partial in the sense that with 
clinical recovery the laboratory tests remain par- 
tially or less strongly positive. 



Case 117. Annie Martin was a charwoman, 37 years of 
age. She had applied for relief at a general hospital, to which 
she was admitted on the suspicion of nephritis; but upon 
admission she became markedly excited and noisy, and spoke 
of seeing angels and hearing God speak to her. As the at- 
tendants were unable to quiet her, she was promptly trans- 
ferred to the Psychopathic Hospital. She maintained that 
she had been sent to the Psychopathic Hospital through the 
spite of the general hospital doctors, and she claimed that 
other people were also attempting to work her harm for the 
purpose of taking her children from her. Visual and auditory 
hallucinations were marked, as was the patient's loquacity, 
irritability, and flight of ideas. However, she seemed entirely 
oriented and her memory appeared to be intact. She was 
able to explain somewhat clearly her supposed condition. 
The voices told her that somebody was after her and that 
her soul belonged to the devil; that she was to be married 
but that her soul was to be damned. These voices probably 
belonged to priests. She was under the impression that she 
was going to be sent to an electric chair and said, " I think I 
am coming to the end and I want a pair of rosary beads 
before the end comes." 

This patient's pupils were markedly unequal and entirely- 
stiff to light and accommodation. Neurologically, however, 
there were no other symptoms. There was a slight trace of 
albumin in the urine and there were no casts. 

The psychiatric diagnosis in this case would off-hand 
undoubtedly be dementia praecox. Yet the stiff pupils are 
almost proof positive of neurosyphilis. If further proof 
were necessary, it is found in the laboratory tests, which 



TREATMENT 383 

showed a positive W. R. of the serum and fluid, with a 
" paretic " gold sol reaction; there were 22 cells per cmm., 
there was excess albumin, and a positive globulin reaction. 
Under intensive antisyphilitic treatment, there was a slow 
improvement. After several months, the patient was en- 
tirely free from mental symptoms; the spinal fluid tests 
became entirely negative except that the gold sol reaction 
has remained strongly positive. 

1. Should treatment be continued in the case of Annie 

Martin in spite of the clinical recovery and the negative 
tests except the gold sol? We would again emphasize 
that it is unreasonable to suppose that a long standing 
case of syphilis can be cured in a period of a few months 
of treatment and while the tests may become negative, 
it would seem foolhardy to stop treatment on this 
account. We do know that in many cases a Wasser- 
mann reaction remaining negative for many months 
may again become positive, indicating that the nega- 
tive reaction did not mean cure but rather the absence 
of the Wassermann bodies in the circulation at the time 
the test was made. 

2. What is the significance of the paretic gold sol reaction 

when the other tests have become negative? As 
previously stated, the gold reducing substance in the 
spinal fluid seems to be different from the substances 
which give the other pathological reactions. We 
should feel in this case that the process which was 
producing these gold reducing bodies had not been 
stopped, in other words, cure was not complete. 

3. Should one make a diagnosis on the " paretic " gold sol 

reaction alone? The so-called paretic gold sol curve 
is not always indicative of general paresis or even of 
syphilis but may occur in non-syphilitic conditions as 
brain tumor, multiple sclerosis, etc. In our experience 
we have seen no case of untreated neurosyphilis in 
which the gold sol alone was positive, that is, in cases 
in which therapy has not changed the findings in the 
spinal fluid. In our experience the gold sol reaction 
has been fortified by one or several of the other tests 
as the W. R., globulin test, pleocytosis. 



384 



TREATMENT 



Some effects of systematic intravenous salvarsan 
therapy in PARETIC NEUROSYPHILIS ('* general 
paresis ") are limited to the laboratory findings 
without clinical improvement. 



Two examples of such limitation are offered: William 
Roberts (ii8) and John Silver (119). 

Case 118. A bank teller, William Roberts, 39, was sent 
to the Psychopathic Hospital for a depression so marked that 
he had become entirely unable to work or care for himself. 
The story was that some money had been left him by his 
uncle, that Roberts could not prove his right to the money, 
and that depression, insomnia, and occasional periods of 
confusion had followed during a period of about five months. 

On admission, Roberts appeared wholly disoriented and 
unable even to give his correct age. Attention could not 
be held, and the patient would slide off into statements like: 
" Oh, I made a mistake, I fooled a lot of people, I have a 
terrible disease, they are going to get it, they are going to get 
me," etc., etc. There was great difficulty in thinking, and a 
marked reaction of fear. This cluster of phenomena certainly 
suggested very strongly the diagnosis of manic-depressive 
psychosis. 

Neurologically, Roberts proved quite negative except that 
the tendon reflexes were very active and the pupils reacted 
somewhat sluggishly to light. The blood serum W. R. was 
negative. No history of syphilis could be obtained; never- 
theless, Roberts kept dropping remarks about the terrible 
disease from which he was suffering. It seemed best to pro- 
ceed to lumbar puncture, and the spinal fluid disclosed a 
positive W. R., globulin. Increased albumin, pleocytosis, and 
" paretic " gold sol reaction. 

The diagnosis of General Paresis was accordingly made. 
During the next year and a half, no improvement was made ; 
a slight speech defect was developed, and tremors of the hand 
and tongue appeared. 



TREATMENT 385 

The effect of treatment is particularly instructive. Only 
after 18 months in the hospital was intensive antisyphilitic 
treatment instituted; but after a few months of this treat- 
ment the W. R. of the spinal fluid had become negative, the 
cells normal in number, globulin absent, albumin present 
only in normal amount. Only the gold sol reaction re- 
mained positive. It is still of a paretic type. Treatment, 
however, did not succeed in altering the patient's mental 
condition in the slightest. At the end of many months of 
treatment, we still confront a man showing marked psychic 
symptoms and a " paretic " gold sol reaction without other 
laboratory signs. 

1. What is the significance of the practically negative tests 

in this case without clinical improvement? One must 
believe that the tests became negative as the result of 
treatment, and that this change in the tests was due to 
the clearing up of some inflammatory reactions which 
were present. This may mean that the syphilis had 
been reduced to inactivity or latency if not cured, or 
at least that there was no activity sufficient to cause 
a positive W. R. in the blood serum, whereas whatever 
activity was present in the brain was in such a region 
that it did not cause any reacting substances to be cast 
into the spinal fluid. This would not mean that there 
would necessarily be any return of function already lost, 
because this may be considered as a permanent loss which 
cannot be compensated for. As to these tests, we now 
feel that the case should remain stationary; that is, 
that no new symptoms will be added. However, we 
believe that it is somewhat premature with our present 
knowledge to make this claim very forcibly, and would 
rather suggest that this case be considered as demon- 
strating an interesting fact, the meaning of which can 
be learned only after a period of years. 

2. Why does the gold sol reaction remain strongly positive 

when all the other tests become negative? As already 
pointed out, above (Case Martin (117)) there is no known 
rule about the disappearance of one or other of the ab- 
normal findings in spinal fluid under treatment, and we 
can at present offer no explanation of this phenomenon. 
It does, however, illustrate how careful we must be in 
drawing any conclusions from tests in cases that are 
being treated. 



386 TREATMENT 



Diminution in the spinal fluid tests may occur 
in treated cases of neurosjrphilis without clinical 
improvement. 



Case 119. John Silver, a man 29 years of age, presented 
classical symptoms of General Paresis: He had a convul- 
sion shortly before his admission to the Psychopathic Hospital, 
his memory was poor, he was only partially oriented, he was 
very euphoric and expansive — thought he had millions, 
that he was the Czar of Russia, and so on. His tendon re- 
flexes were very much increased and there was a marked 
speech defect. The W. R. of both blood and spinal fluid 
were strongly positive; the spinal fluid showed globulin, 
increased albumin, pleocytosis, and a " paretic " gold sol 
reaction. There was, therefore, no question about the 
diagnosis, and the patient was at once put under antlsyphl- 
lltlc treatment. This was continued for five months ; slowly 
the Intensity of the reactions in the spinal fluid diminished. 
At the end of the five months, there was the very slightest 
possible trace of globulin, with a doubtful increase in albumin, 
one cell per cmm., and a mild syphilitic gold sol reaction. 
The W. R.'s In the blood and spinal fluid, however, remained 
strongly positive. There was no mental improvement co- 
incident with the weakening of the spinal fluid tests, and at 
the end of the five months, the patient had a series of con- 
vulsions in which he died. 

This case Is given as a contrast to Case Henry (i 14) in which 
clinical Improvement occurred without diminution In labora- 
tory tests; In the case of John Silver, marked diminution 
in the intensity of these tests had no prognostic signifi- 
cance. This was In keeping with the condition as shown In 
Case Roberts (118) where, while the gold sol was the only test 
to remain positive, the patient did not improve mentally. 

I. What Is the explanation of the lessening of the patho- 
logical elements In the spinal fluid under treatment? 
We have seen that the various findings may occur in- 



TREATMENT 387 

dependently of one another, and we must admit that 
we do not know definitely what it signifies, or why one 
may be present or absent. It has been held by Head 
and Fearnsides that the findings in the spinal fluid 
represent conditions in the spinal cord and spinal 
meninges, or at the base of the brain only, and not 
conditions elsewhere. This is in keeping with our 
finding that the gold sol reaction in the spinal fluid 
post mortem very often differs from that in the ven- 
tricular fluids or cerebral, subdural, and subpial fluids. 
And further, we have found that during life the findings 
in paresis in the spinal fluid may differ markedly from 
those in the third ventricle, and that the change in the 
fluid in these two areas under treatment may not occur 
simultaneously. 



388 



TREATMENT 



Systematic intensive treatment of PARETIC 
NEUROSYPHILIS ("general paresis"), including 
intraventricular injections of salvarsan, may en- 
tirely fail. 



Case 120. James McGinnIs, aged 39, came to the hospital 
on a stretcher, semi-conscious, moaning, unable to reply to 
questions ; there were signs of a right hemiplegia. 

The next day, McGinnis cleared a little and became able 
to utter a few words. His wife said that he had been en- 
tirely well up to four years ago. At that time he was struck 
in the eye by the head of a hammer that flew off the handle. 
Diplopia had developed, but disappeared. 

Only two years later did a marked change appear. McGin- 
nis became careless as to personal appearance. Seemed 
absent-minded, apathetic and drowsy; he would fall asleep 
in his chair or while at work. He lost his position and be- 
came apprehensive, making not very strenuous efforts to 
find work, and finally consulted a physician. The physician 
told him that he had a sluggish liver and gave him calomel. 

Six months later, McGinnis was restored to his position 
as foreman, and his work remained satisfactory for some six 
months. Then (about six months before coming to hospital), 
his speech became slow and somewhat unintelligible. He 
quit work, saying that his speech was going from him and 
that he might be considered to be drunk. His memory 
grew rapidly worse. There was improvement after a vacation 
and he returned to work, but continued to be ataxic, com- 
plained of vertigo, and fell down several times, though 
without loss of consciousness. On the very day of his ad- 
mission to the hospital, in attempting to get out of bed, he 
fell, and psychotic symptoms at once appeared. There 
was slight improvement again with entire disappearance of 
all paralysis after a few days, a slow clearing up of the speech 
disturbance, and a certain return of memory. 

Physically, there was little to note. Neurologically, the left 






TREATMENT 389 

pupil failed to react to light. The tendon reflexes were all 
very active, and more active on the left side. Other abnor- 
mal reflexes were absent. Improvement continued for a num- 
ber of weeks, but the patient never recovered from his speech 
defect, and his memory remained impaired. Irritable at 
times, McGinnis was for the most part very happy and sure 
he would get well. The W. R. of the blood serum was 
negative, but the spinal fluid reaction was strongly positive, 
even down to o.l cc. The globulin and albumin amounts 
were excessive. There was a "paretic" gold sol reaction. 
There were 7 cells per cmm. The diagnosis of General 
Paresis was made. 

Intravenous injections of salvarsan, arsenobenzol or diar- 
senol were made, and intramuscular injections of mercury, 
and potassium iodid by mouth were given. No real im- 
provement occurred after a certain initial betterment; the 
spinal fluid yielded no changes. Diarsenolized serum ac- 
cording to the Swift-Ellis technique was then injected into 
the third ventricle. Under this treatment also there was no 
change for the better over a period of several months. The 
patient died suddenly after a series of convulsions, apparently 
from paralysis of respiration. 

I. What are the causes of hemiplegia and confusion or 
unconsciousness? We must consider epilepsy, brain 
tumor, cerebral thrombosis, cerebral hemorrhage, mul- 
tiple sclerosis, cerebral spinal syphilis, and general 
paresis. 



390 



TREATMENT 



MILD TREATMENT, often thought "adequate," 
MAY FAIL, WHEN INTENSIVE TREATMENT 
PROVES SUCCESSFUL. 



Case 121. Arthur Bright, a printer, had acquired syphilis 
in his 49th year, some six months before examination. He 
had been treated during these six months by three injections 
of salvarsan, injections of mercury, and mercury by mouth. 
He had been apparently cured until about a month before 
admission. He had fallen without warning from his chair 
in a convulsion accompanied by unconsciousness, which lasted 
about two hours. The patient had since been feeling rather 
peculiar. For instance, time seemed to flow too rapidly. 
Sometimes the patient had had difficulty in talking. 

Physically, nothing abnormal could be found either in 
general condition or neurologically. The patient was, how- 
ever, incontinent. Mentally, he was apathetic and unalert, 
even paying no attention to his outside physician when he 
came to visit him. 

The diagnosis of cerebrospinal syphilis already suggested 
by his history was confirmed by the laboratory tests, which 
showed a positive serum and spinal fluid W. R., paretic 
gold sol reaction, 41 cells per cmm., an excess of albumin, 
and a positive globulin test. 

1. What is the prognosis in cerebrospinal syphilis in the 

early secondary stage? The prognosis appears very 
good provided that intensive treatment be given and 
provided that no vascular insult or other focal destruc- 
tive lesion occurs before treatment has had time to do 
its work. 

2. Why did not the " effective " (?) treatment for the syphilis, 

dating from the primary lesion, succeed in staving off 
the cerebrospinal syphilis? It remains a question 
whether the treatment by three injections of salvarsan 
was efficient in this particular case. Of course, it may 
prove true that no treatment whatever in the present 
stage of knowledge will stave off cerebrospinal symp- 
toms in certain cases. 



TREATMENT 39 1 

Treatment: Bright was given intravenous injections of 
diarsenol twice a week, with occasional injections of mercury 
salicylate. After two weeks, the patient seemed markedly 
improved, and continued to improve rapidly. He was symp- 
tomatically well at six weeks. The spinal fluid had then 
become negative, although the serum W. R. had remained 
positive. 

After discharge from the hospital, Bright returned to his 
work, but continued to take the diarsenol treatment weekly, 
and two months later the serum W. R. became negative. 

Small injections of diarsenol at intervals of a month were 
continued, and Bright remained perfectly well for four 
months, when a peculiar seizure developed and lasted for 
several hours. This seizure consisted in a sort of somnam- 
bulism in which Bright stood up at a table, making marks 
on paper, and could not be persuaded to desist. After this 
seizure. Bright re-entered the hospital, again showed no 
mental or physical symptoms and no abnormalities of blood 
or spinal fluid. 

3. What is the explanation of this seizure? It is possibly 
due to a small vascular insult, for which potassium 
iodid may be suggested with precautions as to hygiene 
and continued observation. He has since remained 
entirely well. 



392 



TREATMENT 



Another example where MILD MEASURES 
(though conceived to be "adequate") SEEMED 
TO BE LEADING TO FAILURE; INTENSIVE 
THERAPY SUCCESSFUL. 



Case 122. Levi Morovitz, a waiter, 39 years of age, came 
to the hospital with evidences of an old left hemiplegia, in- 
cluding the left side of the face (there was a left-sided Bab- 
inski, Gordon, and Oppenheim, and all the reflexes were 
fairly active; sluggish pupil reactions, Rombergism, and 
speech defect). Morovitz was much depressed, very slow 
in thinking processes, had a marked memory disturbance in 
general and apparently much deterioration mentally. 

A history was obtained to the effect that Morovitz had 
acquired syphilis at about 33, but that he had received 
practically continuous treatment ever since at a dispensary. 
He had, in fact, received four injections of salvarsan a year 
before coming to the hospital. Of late, Morovitz had become 
much more cheerful and talkative, imagining he could do 
great things if he had money. He had begun to eat very 
rapidly and to be very nervous. His feet had begun to drag; 
a distinct speech defect developed, but from this he had re- 
covered. About six weeks before entrance, Morovitz had a 
shock, which left him with the left hemiplegia above men- 
tioned and with considerable headache. 

Even while the preliminary examination was being per- 
formed, Morovitz developed a minor seizure without loss of 
consciousness. First came severe pain over the frontal 
region, which grew In severity so that the patient held his 
head in his hands. A bit later, twitching movements began 
in the thumb and In the fingers of the left hand, and the 
small muscles of the extensor group of the thumb and third 
finger showed contractions. These contractions grew more 
general and the excursions of the fingers greater, until finally 
every finger of the left hand became Involved, whereupon 
movements of the same sort, though of smaller amplitude, 



TREATMENT 393 

began in the other hand. Finally the left arm began to jerk 
with alternate contractions of the biceps and triceps. The 
whole seizure lasted more than five minutes. During the 
seizure there was dizziness and pain in the head, chiefly on 
the right side. 

Diagnosis : The attention is at once arrested by the data 
of the seizures described. It appeared that we had to as- 
sume an irritation of the right side of the brain, possibly due 
to vascular disease, or to brain tumor, or perhaps to syphilis. 
The shock with residual hemiplegia would be consistent 
enough with any of these diagnoses. However, the history 
seemed somewhat long for brain tumor. Nor were there 
any definite symptoms of intracranial pressure. "Adequate" 
treatment unfortunately does not rule out syphilis. The 
comparatively early age (39) of the patient makes it difficult 
to explain the vascular disease except on the basis of syphilis. 
Add to the hemiplegia the euphoria and grandiose ideas of a 
year's duration, and we arrive at a diagnosis of neurosyphilis, 
probably Paretic Neurosyphilis. 

The laboratory tests showed the W. R. of the serum and 
spinal fluid positive, 80 cells per cmm. in the fluid, large 
amounts of globulin and albumin, and a " paretic " type of 
gold sol reaction. 

To be sure the Jacksonian seizure is not especially charac- 
teristic of paretic neurosyphilis, and even suggests a local 
irritation in the motor area, such as a localized meningitis, 
possibly of a diffuse gummatous nature. 

This patient was put on intensive antisyphilitic treat- 
ment, namely, salvarsan twice a week and injections of 
mercury. He recovered rapidly. After a few months he 
left the hospital, and after treatment had continued for a 
year, he resumed his work by which time both blood and 
spinal fluid had become negative. 

It must be recalled that this patient had from the time of 
his infection what has been considered good antisyphilitic 
therapy, in spite of which he developed after a period of 
years, the symptoms and signs of neurosyphilis in its most 
dangerous form. The conclusion must be drawn that 
however good such treatment is for the majority of cases, it 



394 



TREATMENT 



was insufficient for Morovitz. That the early failure to cure 
was not due to any "drug- fastness" of the spirochete or to any 
peculiarity of strain is proved by the result of more vigorous 
antisyphilitic treatment which caused an apparent if not a real 
cure. With our modern methods of treatment checked by 
Wassermann reactions and spinal fluid examinations, treat- 
ment is given according to the needs of the individual patient 
rather than according to general preconceptions. We have 
reason to believe that under these conditions there will be 
fewer cases developing late symptoms on account of in- 
sufficient treatment given even to patients who are willing 
to co-operate to the last degree. 

The fact that Morovitz had no apparent symptoms for 
several years led to rather desultory treatment chiefly in the 
form of mercury by mouth. Previous to the time when the 
W. R. and lumbar puncture were available, the physician had 
no exact means of determining cure except the non-appearance 
of symptoms. But a period of years of quiescence before 
the outbreak of symptoms referrable to the involvement of 
the nervous system is characteristic of syphilis. With this 
knowledge in mind it is evident that today the care of a 
syphilitic patient must be guided, in part at least, by examina- 
tions of the spinal fluid and W. R. 



TREATMENT 395 



Salvarsan treatment may even occasionally be of 
value in simple FEEBLEMINDEDNESS due to 
congenital syphilis. 



Case 123. The somewhat unattractive Robert Matthews 
was brought, at 5 years of age, to the hospital for backward- 
ness of mind. It appears that the patient was born at term, 
with instruments, that he began to talk at a year, and to 
walk at 13 months, but that in point of fact, he had not 
talked intelligibly to date. Robert had never played with 
other children and is regarded by his parents as backward. 
In fact, Robert's sister — a year his junior — is much 
brighter. Robert had had scarlet fever but without sequelae. 

Examination by the Binet scale showed that, although he 
is actually 5I years, he graded by the Binet scale at 4 and 
was regarded as feebleminded. 

The physical exeimination showed a general adenopathy 
and prominent frontal bosses. In the study of the family 
history in the search for an etiology for the evident feeble- 
mindedness, little or none could be found. There were no 
miscarriages or stillbirths; the parents were living and well. 
There was only the one sister above-mentioned, who is 
brighter than Robert. 

The advantage of a routine W. R. is here well shown, for 
the W. R. in the serum was positive. 

1. What is the prognosis of cases of syphilitic feebleminded- 

ness? It would appear that every case is an individual 
problem. 

2. What is the effect of treatment? Robert Matthews was 

given mercury protoiodid ^ gr., three times a day, by 
mouth, for three months. The protoiodid was followed 
by ten injections of salvarsan, average: 0.15 gram, 
during six months. At the end of this period, the W. R. 
in the blood had become negative. A re-examination by 
the Binet scale, when Robert was 6y^2 years of age, 
showed him to grade at 5f, so that one might conclude 
that Robert had shown more mental progress in a year 
than he had previously. 



396 TREATMENT 

Note: The patient's sister, 4 years of age, is attractive 
and bright, measuring beyond her actual age according to 
the intelHgence tests. However, the girl was found to have 
a positive W. R. It may be that Robert and his sister 
illustrate the hypothesis of Mott: that the syphilitic virus 
becomes less potent as the years go on, and that the younger 
children in the family are less affected than the older. How- 
ever, in our series, there are a number of instances in which 
this hypothesis is not substantiated. 

3. What is the share of syphilis in the production of feeble- 
mindedness? The percentage of syphilitic cases found 
in institutions is not high. A variety of cases have 
been proved to be congenitally syphilitic in the absence 
of a positive serum W. R. 

Fernald* has charted a comparison of cases diagnosti- 
cated "moron" (that is, feeblemindedness proper, in the 
narrower English sense) and "imbecile." Fernald says that 
the morons have, as a group, many more bad family histories 
than have the imbeciles, to quote — "Only 70% of the 
[imbecile] group have bad family histories. This at first 
seems surprising, but when we consider that more of our 
syphilitic, traumatic, and sporadic cases tend toward the 
lower end of the feebleminded group, and when we remember 
that with such cases there is often a seemingly normal family 
tree, the drop in the curve appears logical." 

The situation with the idiots, of whom only 38 came into 
Fernald's study, was similar; 12 out of 38, or 32%, of idiots, 
had good family histories. On these figures, how unfortu- 
nate it would be to dub feeblemindedness hereditary! It is 
true, however, that 68-70% of the idiots and imbeciles, 
judging by W. E. Fernald's intensive study, do have bad 
family histories. 

Goddardt states that of all the causes of feeblemindedness, 

* Fernald, W. E. Standardized Fields of Inquiry for 
Clinical Studies of Borderline Defectives. Mental Hygiene, 
Vol. I, No. 2, April, 1917. 

t Goddard, H. H., Feeblemindedness, its Causes and 
Consequences, 1914. 



TREATMENT 397 

there is perhaps none for which there is less evidence than 
syphiUs. Goddard found syphiHs in 27 of his intensively 
charted cases of feeblemindedness, that is, in 9% of all his 
charts. He finds the majority of the syphilis cases occurring 
in relatives of the feebleminded to be in the hereditary group ; 
for example, of 164 charts in the hereditary group, 17, or 10%, 
showed syphilis. In 34 charts in a group termed "probably • 
hereditary" 3, or 9%, showed syphilis. Of 37 charts in the 
group termed "neuropathic" 4, or 11%, showed syphilis, 
whereas in 57 "accident" and 8 "no cause" groups, there 
were but 2 (4%), and one, or 13%, showing syphilis. How- 
ever, Goddard concedes that much more careful studies are 
necessary if we are to give an exact evaluation of syphilogenic 
feeblemindedness. 

The first ten of the Waverley Anatomical Series are shortly 
to be described in a forthcoming publication.* Of these ten 
cases, four showed some slight evidence of chronic inflam- 
matory changes, indicating the possibility of a syphilitic or 
similar infectious condition. These cases, be it remembered, 
were not cases of juvenile paresis, but cases of what, for the 
lack of a better name, may be called "ordinary" feeble- 
mindedness. 

If all or any of these processes are syphilitic, the syphilis is 
virtually extinct. The cases had not been treated for syphilis 
and were not regarded as syphilitic, though several of them 
showed a few stigmata somewhat suggestive of syphilis. The 
anatomical conclusion at this time is still doubtful. 

As in the text case, the hypothesis of syphilis as a direct 
cause for simple feeblemindedness must be entertained for a 
few cases. In any event, it would not seem logical to let 
any institution for the feebleminded run without a Wasser- 
mann analysis of the population. In addition to the Wasser- 
mann data from the blood serum, osteological data from the 
X-ray have proved of occasional value for syphilis diagnosis 
in this as in other groups. 

* W. E. Fernald and E. E. Southard. Waverley Research 
Series in the Pathology of the Feebleminded. Proceedings 
of the American Academy of Arts and Sciences, 1 91 7. 



'Within the gates of Hell sat Sin and Death." 

Paradise Lost, Book X, Line 230. 



VI. NEUROSYPHILIS AND THE WAR. 

Although the American toll of war syphilis has not yet 
begun and although the crop of neurosyphilis due to war 
infections may not arrive until the mid or late twenties of 
the century (witness German experience in the eighties of 
the last century), it seems proper here to give a number of 
abstracts re neurosyphilis as it has developed in the war. 
Available reports from English, French, and German sources 
have been levied upon for the years 19 14-16. 

It is clear that all the armies have had their share of 
neurosyphilitics, some clearly diseased before enlistment, 
some developing symptoms as a result of training, stress, 
or shock, others hastened or made worse by war conditions. 

There are important questions of pension, retirement, and 
compensation for neurosyphilitics. No previous war has 
had the benefit of the Wassermann reaction and other exact 
tests bearing upon the nature, progress, and curability of 
neurosyphilis. 

That we shall have our fill of pension and other problems 
can already be seen from continental reports. Thiblerge,* 
for example, states that syphilis has become a real epidemic 
among the French soldiers and mobilized munition workers. 

Hechtf of Austria claims that no less than an equivalent 
of 60 army divisions have been temporarily withdrawn from 
fighting on the Teutonic side for venereal diseases. He 
commends Nelsser's Idea that salvarsan and mercury should 
be given In the trenches. While hundreds or thousands of 
Austrlans are sick with syphilis, sound and healthy men are 
being shot down In their stead. The diagnosis of syphilis, 
according to Hecht, ought to be a signal for sending the men 
to the front. He makes even the somewhat bizarre sugges- 

* Thiblerge. La Syphilis dans I'armee, 191 7. 
t Hecht. Wien. klin. Woch., xxix, 51. 
399 



400 NEUROSYPHILIS AND THE WAR 

tion that special companies of syphilitics should be formed, 
for convenience of treatment, on the firing line. 

Not only is the syphilis problem in the army of importance 
to the military authorities, but also to the civil population, 
and perhaps to them a greater problem. With the great 
increase of venereal disease that is the result of the conditions 
of army life in war time, there will be a considerable percent- 
age of cases developing neurosyphilis a number of years after 
discharge from the army, but caused by the infection acquired 
during service. In addition many men will bring the disease 
back to America in an infectious stage and spread it. We 
would advocate that the names of all soldiers who had 
acquired syphilis and were not considered cured at time of 
discharge should be given to health organizations in their 
home states that they may be given further care. 

These practical and several theoretical questions are 
raised by the following fourteen cases which we have con- 
densed from their sources. 



NEUROSYPHILIS AND THE WAR 4OI 



A tabetic lieutenant "shell-shocked" into paresis? 
Case from Donath of Vienna. 



Case A.* An apparently competent German professor in 
an intermediate school, a lieutenant of infantry reserves, 33 
years old, on the 17th August, 1914, was stunned for a while 
by the shock of a cannon-firing 25 feet away. Urination 
became difficult. Headaches and limb pains ensued, with 
paralysis of fingers, gastric troubles, forgetfulness especially 
for names, insomnia, and general scattering of mental facul- 
ties. 

Neurologically, the pupils were irregular, left larger than 
right; Argyll- Robertson reaction. Right knee-jerk livelier 
than left. Achilles reactions absent. Slow and dissociated 
pain reactions in feet, lower thighs and lower quarter of upper 
thighs, with hypalgesia or analgesia. Station good; gait 
steady. Mentally depressed, slow of thought. Speech poor 
and of indistinct construction (mild dementia). Calculation 
ability poor. No pleasure in work. 

Wassermann reaction of serum weakly positive. 

It seems that for a year the patient had been subject to 
spells of anger. He was irritated by his wife who had been 
nervous since an earthquake. 

On the occasion of the earthquake, 1911, the patient himself 
had had a spell of difficulty with urination. The spell had 
lasted two or three months. The patient had had a chancre 
in 1902, "cured" in four or five weeks with xeroform. In 
1908, when about to marry, he had had six mercurial inunc- 
tions. 

I. Is this a case of traumatic paresis? From the some- 
what meagre account it would appear that Donath's 
lieutenant should rather be termed "shell-shock pare- 
sis," in the sense of a paretic neurosyphilis liberated by 

* Donath. Beitrage zu den Kriegsverletzungen und -er- 
krankungen des Nervensystems. Wiener klin. Wchnschr., 
No. 27-8, 1915. 



402 NEUROSYPHILIS AND THE WAR 

shell-shock (using shell-shock in the sense of a shock 
without direct brain injury). 

2. What compensation is due such a man as Donath's 

lieutenant? The ordinary principles applicable to 
traumatic paresis are not here in point, since no 
symptoms pointing to trauma of brain ever super- 
vened. See discussion under Case G. 

3. How frequent is paresis in armies? R. L. Richards in 

White and Jelliffe's Treatment of Nervous and Mental 
Diseases writes as follows (of course concerning peace 
times) : 

"The French estimate that paresis cases are 7 per 
cent of all their military cases. The German estimate 
is 6.6 per cent. In our own army at the Government 
Hospital for the Insane, of 490 cases of mental diseases 
among officers and enlisted men, 37, or 7 per cent, were 
paresis. During the Russo-Japanese War, in the 
Russian Psychiatric Hospital at Harbin, the percentage 
of paresis was 5.6 per cent among the cases developing 
at the front." 



NEUROSYPHILIS AND THE WAR 403 



A French soldier "shell-shocked" (also burial) 
into incipient tabes dorsalis? Case from Duco and 
Bliun of Paris. 



Case B.* A French soldier was buried by effects of shell 
explosion September 8th, 1914. He sustained no wound or 
fracture. 

Incontinence of urine developed. Anesthesia of penis and 
scrotum. Reflexes absent; pupils sluggish. Wassermann 
reactions suspicious. 

The diagnosis tabes dorsalis incipiens was made (hema- 
tomyelia of conus terminalls eliminated). 

The patient was estimated to be "40% incapacitated," 
according to the French " echelle de graviW of conditions. A 
full pension would not be justified in the opinion of the 
French authors. 

I. Is there evidence of an increase or exacerbation of 
tabes dorsalis in the war? Bimbaum,f reviewing 
German war neurology, quotes Weygandt as believing 
that the war has probably had to do with the produc- 
tion of both tabes and paresis in many instances. 
Other cases, however, have merely been made worse 
by the war stress. Thirdly, there are cases in which 
the war stress has done no harm whatever. Westphal 
has seen both tabes and paresis develop in men who 
had never before shown any mental or physical symp- 
toms whatever, and accordingly, Westphal must be 
counted among those who regard war stress as a liberat- 
ing factor for these diseases. Redlich and Donath are 
cited in the same connection. (The case of Donath is 
the case presented above as Case A.) 

A very interesting claim was made by Cimbal to the 
effect that he found many examples of paresis develop- 
ing In the early period of the war, particularly in Novem- 
ber and December, 1914. Later, according to Cimbal, 
cerebrospinal syphilis and tabes became more prevalent. 

* Duco et Blum. Guide pratique du Medecin dans les 
Expertises medicolegals mllitaires. Paris, 191 7. 

t BIrnbaum. Kriegsneurosen und -psychosen auf Grund 
der gegenwartlgen Kriegsbeobachtungen : Sammelbericht. 
Z. f. d. ges. Neurol, u. Psychlat., Bd. XII, H. i, 1915. 



404 NEUROSYPHILIS AND THE WAR 



Neurosyphilis in a Gennan recruit, possibly AG- 
GRAVATED ON military SERVICE. Pension not 
allowable. Case from Weygandt. 



Case C* A German, long alcoholic and thought to be 
weakminded, volunteered, but shortly had to be released from 
service. He began to be forgetful and obstinate, cried, and 
even appeared to be subject to hallucinations. The pupils 
were unequal and sluggish. The uvula hung to the right. 
The left knee-jerk was lively, right weak. Fine tremors of 
hands. Hypalgesia of backs of hands. Stumbling speech. 
Attention poor. 

It appeared that he had been Infected with syphilis in i88l 
and in 1903 had had an ulcer of the left leg. 

The military commission denied that his service had 
brought about the disease. In the phrase of the Canadian 
Pension Board the German commission would probably have 
rendered a report "aggravated on service," not "by service." 
(See Canadian cases D, E, and F.) 

1. Has paresis increased in the war? Both French and 

German figures controvert the claim. Marie, for 
example, found not a single paretic amongst the skull 
injury cases at the Salpetrlere. Most authors are 
found demonstrating cases which they clearly regard as 
in some way produced or unfavorably influenced by 
the war. There seems, therefore, to be a little Incon- 
sistency between the general statement that paresis has 
not Increased In the war and the somewhat frequent cases 
described as occurring in and modified by the war. 
However, Bonhoeffer, on the basis of nine months' war 
experience, also holds it to be probable that paresis is 
no more frequent in the field than in the home popula- 
tion. 

2. Is the old syphilitic especially liable to break down under 

war conditions? According to Richards, Shalkewicz 
says that in the Russo-Japanese war paresis was noted 



* Weygandt. Kriegselnfliisse und Psychiatric. Jahres- 
kurse f. arztl. Fortbildung, Malheft, 191 5. 



NEUROSYPHILIS AND THE WAR 405 

especially among the officers and non-commissioned 
officers, and that it was undoubtedly hastened in its de- 
velopment by war conditions. Steida says that while 
ordinarily we find paresis developing twelve to twenty 
years after the primary sore of syphilis, in these cases it 
developed in five to ten years after the primary sore. 
Some of the cases progressed with unusual rapidity. It 
was also noticed that among soldiers from the front, 
under treatment, evidences of syphilis were present in 
20%, while among the other soldiers under treatment, 
evidences of syphilis were present in 1.6%. Undoubt- 
edly the old syphilitic is especially liable to break 
down under war conditions. 

But, on the whole, the German authors in this war 
find no evidence favoring Steida's claim of the hastened 
post-infective outbreak. 
How did it come about that the efficient German system 
permitted this alcoholic and weakminded syphilitic to 
enter the army? As will be seen, he was a volunteer. 
In general, the German system has been supplied with 
army surgeons who have been trained, not by brief and 
"brush-up" courses, but by longer periods, sometimes 
two years in duration. 



406 NEUROSYPHILIS AND THE WAR 



Syphilis contracted before enlistment, "AGGRA- 
VATED BY SERVICE." Canadian case, courtesy 
of Dr. J. L. Todd, Canadian Board of Pension 
Commissioners. 



Case D. A laboring man, 42, who always strenuously 
denied syphilitic infection, proceeded to France eight months 
after enlistment. He had not been in France three weeks 
when he dropped unconscious. He regained consciousness, 
but remained stupid, dull in expression, and with memory 
impaired. His speech was also impaired. There was dizzi- 
ness and a right-sided hemiplegia. 

He was confined to bed four months and was then 
"boarded" for discharge. 

Physically, his heart was slightly enlarged both right and 
left ; sounds irregular ; extra systoles ; aortic systolic murmur 
transmitted to neck; blood pressure 140:40. Precordial 
pain, dyspnoea. 

Neurologically, there was a partial spastic paralysis of the 
right thigh which could be abducted, could be flexed to 120°, 
and showed some power in the quadriceps. There was also 
a spastic paralysis of the right arm, but the shoulder girdle 
movements were not Impaired. There was a slight weakness 
on the right side of the face. There was no anesthesia 
anywhere. 

The deep reflexes were increased on the right side, Babinski 
on right, flexor contractures of right hand, extensor contrac- 
tures of right leg, abdominal and epigastric reflexes absent, 
pupils active, tongue protruded in straight line. 

Fluid: slight increase in protein. W. R.+ + + 

The Board of Pension Commissioners ruled that the condi- 
tion had been aggravated hy service. (See Case E, "aggra- 
vated on service.") 

I. In view of the fact that the majority of the cases here 
abstracted happen to be in common soldiers, is there 
any evidence bearing on relative incidence in officers 
and men? Quoting R. L. Richards: 



NEUROSYPHILIS AND THE WAR 407 

"The percentage of paresis cases among officers alone 
is variously estimated from 50 per cent in the German 
army (Stier) to 58.9 per cent in the Austrian army 
(Drastich). Since paresis is a disease of more advanced 
life, it is but natural that the percentage of paresis 
among officers, non-commissioned officers, and older 
soldiers should be higher than among the whole military 
body, where the average age is, as we have seen, well 
below thirty years. Hence the above figures do not 
mean a greater prevalence of syphilis among those, 
classes, but that we have no means of knowing how 
many of the others develop paresis. If anything it 
shows that these 'soldiers by calling,' have a more 
stable mental make-up, since they succumb chiefly to 
an exogenous toxin." 

Rayneau at the 19th Congress of French Alienists 
and Neurologists at Nantes in 1909, discussing the 
insane of the army from a medico-legal point of view, 
states that the most frequent mental disease amongst 
officers and soldiers is general paresis. At least, this 
disease is the most frequent basis of invaliding, retire- 
ment, or placing in the inactive list. He states that 
French and foreign statistics are at one upon this 
matter, quoting Christian as finding 32% among the 
soldiers interned at Charenton; Garnier at Dijon, 
59%; Meilhon at Quimper, 42% and Talon at Mar- 
seilles, 33.8%. Grilli found 31 of 40 officers interned 
in Florence, Sienna and Milan victims of general 
paresis. Stier's German statistics indicate about 50%. 
Rayneau himself found 16 of 20 officers paretic and 17 
out of 27 subalterns and gendarmes. 

The Neurological Society of Paris held a conference 
December 15, 191 6, with the chiefs of the neurological 
and psychiatric military centres of France, and dis- 
cussed a variety of questions concerning invaliding, 
incapacity, and compensation in neuroses and psy- 
choses of war. Dupre dealt especially with the psy- 
choses of war as caused by trauma, strain, infectiori, 
and intoxication. General paresis is regarded by 
Dupre as the most important of the dementias found in 
the army. The medico-legal point of view is, of course, 
that general paresis is necessarily related to an old 
syphilis, but its late development leads to misinterpre- 
tations as to its probable cause, both by the family and 
friends and even by magistrates. The war acts in 
the French nomenclature as an agent revelateur or as an 



408 NEUROSYPHILIS AND THE WAR 

agent accelerateur. Although its cause is prior and 
exterior to the war, general paresis in a majority of 
cases is brought out {reveie) by the lack of adaptability 
of the general paretic to the novelty and difficulties of 
his surroundings and duties in war. Trauma, strain, 
and alcohol in a certain number of cases accelerate the 
progress of a general paresis. The aggravation of 
paresis is produced by these same factors, but especially 
by violent cerebral trauma. According to Dupre, the 
Val-de-Grace statistics show that the number of pa- 
retics has not been increased by the war. Medico- 
legally, the victim of general paresis, like the victim of 
traumatic or infectious chronic mental disorder, may 
be assigned an incapacity of from 50 to 100%, and 
these patients are invalided under Reforme No. I, — a 
permanent invaliding. 

Lupine of Lyons also discusses the compensation 
question in general paresis. Lepine thinks that, al- 
though syphilis is indispensable in paresis, yet the truth 
is that syphilis plus something else unknown to us is 
responsible for general paresis. This something else is 
neither a special kind of virus nor is it a particular kind 
of prepared soil alone. Trauma, physical, intellectual, 
and moral strain, and insomnia are the factors to which 
he calls special attention as adjuncts in the production 
of general paresis. As to the responsibility of the State 
for the production of general paresis, according to 
Lepine, the maximal responsibility should be 40% on 
account of the very considerable predisposition to paresis 
created by pre-existent syphilis. 

Marie remarked that, although there had been 
thousands of head cases at the Salpetri^re, there had 
not been a single case of general paresis. Dupre agreed 
with Marie that trauma was not a frequent etiological 
factor; strain and alcohol were more important. The 
Society agreed that in exceptional cases, where an 
encephalic trauma could be regarded as accelerating 
or aggravating the disease, the degree of incapacity 
might be set at from 10 to 30 per cent. 



NEUROSYPHILIS AND THE WAR 409 



Syphilis contracted before enlistment, "AGGRA- 
VATED ON SERVICE." Canadian case, courtesy 
of Dr. J. L. Todd, Canadian Board of Pension 
Commissioners. 



Case E. A laboring man, 44, acquired syphilis at a time 
unknown. Ten months after enlistment this man developed 
symptoms on the firing-line. He was inattentive, irrational, 
incoherent. The diagnosis was then "mania." 

There were, however, scars at angle of mouth and on lower 
lip. Occipital glands were palpable, fine tremor of hands. 
TheW. R. was + + +. 

Later the patient became violent, destructive, untidy, 
disoriented. Auditory hallucinations are recorded. 

He was "boarded" for discharge five months after the first 
symptoms. The board agreed that these symptoms would 
have appeared in civil life. In view of a difference of opinion 
as to the part played by stress of service, his condition was 
set down as ''aggravated on service " (not, it will be noted, hy 
service, see Case D). 

I. Under what conditions should pensions be awarded for 
disability resulting from venereal diseases? According 
to a personal communication from Dr. J. L. Todd, 
Chairman of the Board of Pension Commissioners for 
Canada, pensions are awarded for all disabilities appear- 
ing during service, unless they can be shown certainly 
to be due to the men's own fault and negligence. It 
would appear that during service covers both aggra- 
vations by and on service. There remains some doubt 
as to whether contraction of venereal disease constitutes 
negligence. 

Z. What have been conditions in the small inactive American 
army of the past? Richards has made a study of 
statistics at the Government Hospital for the Insane, 
Washington. 

"The leading features of this mental disease were 
well exemplified in our cases the past year. They 
formed 7.5 per cent of the total number. They aver- 
aged forty years of age, and Ziehen says 80 per cent of 



4IO NEUROSYPHILIS AND THE WAR 

all cases are in the fourth or fifth decade of life. They 
averaged ten and a half years' service, which would 
indicate that the military life was their calling. Only 
one had any serious hereditary defect. Stigmata of 
degeneration were infrequent, averaging only two for 
each case. 66 per cent had good schooling, considering 
their opportunities. Physical signs were frequent in 
each case. Only one showed normal light reaction. 
Ziehen says the light reaction is retained in only 20 
per cent of the cases. Patellar reflex was absent in one 
case and normal or exaggerated in five. The speech 
defect was slight in four cases. Other physical signs 
were present in the usual proportions. Memory de- 
fects existed in all the cases. In four the onset was 
with excitement. One began with a character change 
as the most marked feature. In only two were the 
transfer diagnoses correct. One, beginning as a quiet 
dementia, was diagnosticated paralysis agitans, be- 
cause of a marked tremor. One was excited and 
euphoric and was called a manic-depressive psychosis. 
One with an obscure onset was diagnosticated as a 
neurasthenic. The other one was first observed in this 
hospital. The physical signs should have led to a 
correct diagnosis in each of these cases." 



NEUROSYPHILIS AND THE WAR 4II 



Duration of neurosyphilitic process important re 
compensation. Canadian case, courtesy of Dr. 
C. B. Farrar, Psychiatrist, Military Hospitals 



Commission. 



Case F. A Canadian of 36 enlisted in 191 5, served In 
England, and was returned to Canada In February, 191 7, 
clearly suffering from some form of neurosyphilis (W. R. 
positive in serum and fluid, globulin, pleocytosis 108). 

There Is no record of any disability or symptom of nervous 
or mental disease at enlistment. The first symptoms were 
noted by the patient In May, 1916, six months or more after 
enlistment. The case was reviewed at a Canadian Special 
Hospital, October 11, 1916, by a board of examiners. This 
board reported that: 

"The condition could only come from syphilitic infection 
of three years' standing" (a decision bearing on compensa- 
tion) ; but the general diagnosis remained : 

"Cerebrospinal lues, aggravated by service." 

The picture which the medical board regarded as of at least 
three years' standing was as follows: 

History of Incontinence, shooting pains, attacks of syncope, 
general weakness, facial tremor, exaggerated knee-jerks, 
pupils react with small excursion. Speech and writing dis- 
order, perception dull, lapses of attention, memory defect, 
defective Insight Into nature of disorder, emotional apathy. 

1. Was the conclusion "aggravated by service" sound? 

On humanitarian grounds the victim Is naturally con- 
ceded the benefit of the doubt. But It Is questionable 
how scientifically sound the conclusion really was. 

2. Could the condition come only from syphilitic Infection 

of at least three years' standing? Hardly any single 
symptom in this case need be of so long a standing ; yet 
the combination of symptoms seems by very weight of 
numbers to justify the conclusion of the medical board. 



412 NEUROSYPHILIS AND THE WAR 



Can PARETIC NEUROSYPHILIS ("general 
paresis ") be lighted up by the stress of military 
service without injury or disease? A possible 
example from P. Marie, Chatelin and Patrikios 
of Paris. 



Case G. In apparently good health a French soldier 
repaired to the colors, in August, 19 14, being then 23 years 
old. 

Two years later, August, 1916, symptoms appeared: 
speech disorder with stammering, change of character (had 
become easily excitable), stumbling gait. He became more 
and more preoccupied with his own affairs, grew worse, and 
was sent to hospital in October, 191 6. 

He was then foolish and overhappy, especially when inter- 
viewed. There was marked rapid tremor of face and tongue. 
Speech hesitant, monotonous, and stammering to the point 
of unlntelllglblllty. His memory, at first preserved, became 
impaired so that half of a test phrase was forgotten. Simple 
addition was Impossible and fantastic sums would be given 
instead of right answers; handwriting tremulous, letters often 
missed, others Irregular, unequal, and misshapen. 

Excitable from onset, the patient now became at times 
suddenly violent, striking his wife without provocation. 
After visit at home, he would forget to return to hospital. 
Often he would leave hospital without permission (of course 
the more surprising In a disciplined soldier). 

No delusions were found. 

The serum and fluid W. R. were positive, albumin in fluid, 
lymphocytosis. 

Neurological examination. Unequal pupils, slight right- 
side mydriasis, pupils stiff to light, weakly responsive in 
accommodation, reflexes lively, fingers tremulous on exten- 
sion of arms. 

The patient had, December 5, 191 6, an epileptiform attack 
with head rotation, limb-contractions and clonic movements. 



NEUROSYPHILIS AND THE WAR 413 

1. Should this soldier recover for disability obtainedj^ in 

service? Marie was inclined to think military service 
in part responsible for the development of the paresis. 
Laignel-Lavastine thought so also, but that the 
amount assigned should be 5%-io% of the maximum 
assignable. 

2. What is the duty of the military authorities relative to 

socalled traumatic paresis? Medico-legally speaking, 
Froissart, quoted by Rayneau, states that a victim of 
traumatic paresis may or may not have presented mental 
disorders before the accident, that is, that the paretic 
symptoms may develop out of a clear sky as a result 
of the accident. The accident itself must be of a 
serious nature. The accident must be followed by 
phenomena pointing to brain injury of traumatic 
nature. These phenomena need not be characteristic 
symptoms of general paresis at the outset. The period 
elapsing between the trauma and the supervening con- 
dition of paresis must be occupied without notable 
interruption, at first by phenomena of a purely trau- 
matic nature, later by signs indicating the onset and 
evolution of general paresis. 

The French invaliding process called Reforme No. i 
with pension is granted according to the governmental 
instructions only to officers, subalterns, and soldiers 
whose disease is due to trauma. In view of this 
governmental regulation, the military surgeon must 
write out certificates describing every cranial trauma, 
however slight, which might have a bearing on the 
development of paresis. However, he should not too 
readily admit trauma as a cause of paresis. If a long 
period of quietude, a period in which the trauma itself 
seems to have undergone a complete recovery, super- 
venes, then general paresis should not be reported by 
the surgeon. 

Lepine has recently noted the following features as 
desirable in board reports concerning paretics: nature 
of trauma, length of service, fatigue endured, insomnia, 
date of infection, treatment, W, R. 



414 NEUROSYPHILIS AND THE WAR 



light up a paresis? Example from 
de Massary of Issy-les-Moulineaux. 



Case H. A soldier, 35, was sent to the Centre Neurologique 
with a hospital ticket reading : 

"Neurasthenia, general weakness following intoxication 
by gas." 

The soldier was thought at first to be a neurasthenic. But 
he soon showed signs of more pronounced mental trouble. 
The voice was suspicious. There was a slight irregularity of 
pupils. 

An epileptiform attack occurred, followed by aggravation 
of symptoms. 

Lumbar puncture showed pleocytosis. The W. R. of the 
serum proved positive. 

Yet the evident neurosyphilis, possibly paretic (de Mas- 
sary's diagnosis), was preceded by a neurasthenia and the 
neurasthenia was preceded by "gassing." 

De Massary believes the patient and his family would 
perhaps be justified in believing the condition produced by the 
injury. De Massary is not clear as to the financial deserts of 
the patient. It is not a manifest case of aggravation of ante- 
bellum symptoms, even if it be neuropathologically an in- 
stance of acquired loss of resistance to pre-existent spirochetes 
in body or brain. 

I. What adjuvant factors have been recognized in military 
paresis? Aside from syphilis, Rayneau finds that alco- 
holism, malaria, sunstroke and various intoxications 
serve as causes for paresis. Rayneau points out that the 
apparent integrity of the mind in general paresis may 
be such that they last in the army some time and have 
their oddities ascribed to misconduct or breaches of 
discipline. In fact the Legrande du Saulle called this 
early period in general paresis the medico-legal period, 
showing, as it so often does, thefts, outrages against 
decency, frauds, assaults, exhibitionism and the like. 
To be sure these acts are absurd and infantile and not 
difficult to recognize as of psychotic origin. 



NEUROSYPHILIS AND THE WAR 4I5 



Syphilis may bring out epilepsy in a subject having 
taint. Case from Bonhoeffer, 1915. 



. Case I.* A man of 35 in the Landwehr acquired syphilis 
some time in the summer of 19 14. He was a good soldier, 
passed through several clashes, and was promoted to Unter- 
offizier. 

To understand what followed it must be stated that he had 
been a bed-wetter to 11, had been practically a teetotaler 
(Bonhoeffer's point is perhaps that otherwise epilepsy might 
have developed sooner?), and, when he did drink, vomited 
almost at once, and had amnesia for the period of drunken- 
ness. His father had been somewhat of a drinker. His 
sister had suffered from convulsions as a child. 

February, 1915, the Unteroffizier lost appetite, got head- 
aches, and went to hospital for a time. Upon getting better, 
he was sent on service to Berlin. In a Berlin hotel he had 
his first convulsions and unconsciousness, biting his tongue. 
He was confused for several days, and, when he had become 
clear, had a pronounced retrograde amnesia together with a 
tendency to fabricate a filling for the lost period. 

This retrograde amnesia is uncommon in epilepsy and 
suggests organic disease. No sign of organic disease was 
found on neurological examination. The patient had no 
signs of the epileptic make-up. The serum W. R. was 
negative. On the whole, Bonhoeffer regards the epilepsy as 
"reactive " to the syphilis, as a syphilogenic epilepsy. 

As to the amnesia, it is of interest that alcohol should long 
before have been able to cause amnesia In this man in the 
same way as does now the syphilitic epilepsy. 

I. In view of the fact that this Landwehr man appears to 
have acquired syphilis while on campaign, what is the 
responsibility of the government for treatment? The 

* Bonhoeffer. Erfahrungen iiber Epilepsle und Ver- 
wandtes Im Feldzuge. Monatschr. f. Psychlat u. Neurol., 
Bd. 38, H. 1-2, 1915, 



4l6 NEUROSYPHILIS AND THE WAR 

Canadian authorities, as stated under Case E, are in 
doubt whether contraction of venereal disease con- 
stitutes negHgence on the part of the soldier. It would 
appear to us that where a government does not take 
suitable steps to prevent the acquisition of syphilis by 
the soldiers, the government must assume a measure 
of responsibility for the syphilis incurred. The govern- 
ment's responsibility would be still greater in equity, 
it would appear, if commercial opportunities for the 
acquisition of syphilis are maintained under more or 
less close government supervision or (even as has been 
claimed for certain encampments on our own Mexican 
border) if shelter for illicit sex relations is afforded 
within the limits of a military camp. In a certain com- 
munity, "E," for example, it is claimed by Exner,* the 
district for prostitutes was "situated within the lines of 
military camps and protected and 'regulated' by the 
military authorities." 

But even if the government has no legal responsi- 
bility in this regard, it would be well to consider the 
ultimate results of the syphilis that will probably be 
acquired by great numbers of soldiers under campaign 
conditions. Aside from the ravages of syphilis outside 
the nervous system, it is well known, as Weygandt 
intimates for German conditions, that the aftermath 
of war will be a high proportion of cases of neuro- 
syphilis. 

! Weygandt remarks in his review of the influence of 
the war upon psychiatry, that the opportunity for 
syphilitic infection in the campaign is considerable. 
In the war of 1870, the conditions in this regard were 
extremely unfavorable, and writing in 1915, Weygandt 
remarks that at present there should be a prophylaxis 
against syphilitic infection by the soldiers, which 
prophylaxis should be the most energetic possible. 
Continence on the part of the soldiers and the isolation 
of infected women, with examination by specialists, 
have been advocated by Neisser and by Mendel. In 
the '8o's a great number of cases of locomotor ataxia 
developed in Germany, which were due to syphilis 
acquired by the soldiers and officers in the war of 1870. 



* Exner, M. J., Prostitution in its relation to the army on. 
the Mexican Border, Social Hygiene, Vol. 3, 2, April, 1917. 



NEUROSYPHILIS AND THE WAR 417 



Syphilis in a psychopathic subject. Convulsions 
5 days after Dixmude. Case from Bonhoeffer, 1915. 



Case J.* A soldier in the reserves, 23, was, subsequently 
to his being brought to hospital, described by his wife as a 
rather over-sensitive fellow, who could hardly look at blood 
and was meticulous about the household. He had always 
been subject to headaches, especially after hard work. 
However, he had passed through his military training well in 
191 o, not even having been bestraft. 

He began service in October and fought at Dixmude on 
the 19th. On the 24th in the trench and while being carried 
back, he had several spells of pallor, falling stifif, and then 
having convulsions. Brought finally to the Charite in Berlin, 
he had more spells of sudden pallor, collapse with brief con- 
vulsions, tossings in bed, and absences, post-convulsive head- 
aches, and mild bad humor. 

There were numerous attacks several days apart in the 
first seven weeks. The patient was not of an "epileptic" 
disposition, though he was rather readily dissatisfied. Head- 
aches also occurred without relation to convulsions. 

The serum W. R. was positive. Treatment by mercurial 
inunctions. No further convulsions. Prognosis as to the 
possibility of a constitutional epilepsy unknown. 

* Bonhoeffer, loc. cit. 



41 8 NEUROSYPHILIS AND THE WAR 



SYPHILITIC ROOT-SCIATICA (lumbrosacral 
radiculitis) in a fireworks man with a French artil- 
lery regiment. Case presented from Dejerine's 
clinic by Long. 



Case K. No direct relation of this example of root- 
sciatica to the war is claimed nor was there a question of 
financial reparation. 

There was no prior injury. At the end of March, 191 5, 
the workman was taken with acute pains in lumbar region 
and thighs, and with urgent but retarded micturition. 

Unfit for work, he remained, however, five months with the 
regiment, and was then retired for two months to a hospital 
behind the lines. He reached the Salpetriere October 12, 
191 5, with "double sciatica, intractable." 

There was no demonstrable paralysis but the legs seemed 
to have "melted away," fondu, as the patient said. Pains 
were spontaneously felt in the lumbar plexus and sciatic 
nerve regions, not passing, however, beyond the thighs. 
These pains were more Intense with movements of legs; but 
coughing did not intensify the pains. Neuralgic points could 
be demonstrated by the finger In lumbar and gluteal regions 
and above and below the iliac crests (corresponding with 
rami of first lumbar nerves). The inguinal region was 
involved and the painful zone reached the sciatic notch and 
the upper part of the posterior surface of the thigh. 

The sensory disorder had another distribution objectively 
tested. The sacral and perineal regions were free. Anes- 
thesia of inner surfaces of thighs, hypesthesia of the anterior 
surfaces of thighs and lower legs. The anesthesia grew more 
and more marked lower down and was maximal In the feet, 
which were practically insensible to all tests, Including those 
for bone sensation. There was a longitudinal strip of skin 
of lower leg which retained sensation. 

Position sense of toes, except great toes, was poor. There 
was a slight ataxia attributable to the sensory disorder — 



NEUROSYPHILIS AKD THE WAR 4I9 

reflexes of upper extremities, abdominal, and cremasteric 
preserved, knee-jerks, Achilles and plantar reactions absent. 

The vesical sphincter shortly regained its function, though 
its disorder had been an initial symptom. 

Pupils normal. 

The "sciatica" here afifects the lumbosacral plexus. 
Signs of disorder at one time or other affected the first lumbar 
distribution of the third lumbar and first and second sacral 
nerves. 

As to the syphilitic nature of this affection, there had been 
at eighteen (22 years before) a colorless small induration of 
the penis, lasting about three weeks. There was now evident 
a small oval pigmented scar. The patient had married at 
20 and has had three healthy children. 

The lumbar puncture fluid yielded pleocytosis (120 per 
cmm.). Mercurial treatment was instituted. 

The treatment has not reduced the pains. Long thinks it 
was undertaken too long (six months) after onset. The 
warning for early diagnosis is manifest. There was somehow 
a delay under the medical conditions of the army, 



420 NEUROSYPHILIS AND THE WAR 



Can the " lighting-up " of NEUROSYPHILIS IN 
CIVIL LIFE be induced by the domestic stress of 
war? A possible example from Dr. R. Percy Smith, 
London. 



Case L. A German Jew in London passed into the 
Paretic form of Neurosyphilis shortly after the outbreak 
of war under conditions suggesting that the stress of emotions 
directly or indirectly lighted up the neural process. 

The man was a bank-officer, 52 years old, and married. 
He had lived many years in England and was in fact a 
naturalized citizen. He had been under treatment for 
syphilis by Sir Jonathan Hutchinson, 29 years before, namely, 
at the age of 23. Subsequently, Sir John had given him per- 
mission to marry. 

It proved that for years the man had had fixed pupils, 
absent knee-jerks, and a perforated ulcer of the foot. How- 
ever, there had been no other mental or nervous symptoms 
preventing bank-officer's work. 

At the outbreak of war the man was discharged from the 
bank. He grew worried and sleepless. He began to charge 
himself with sex irregularity. He went down to the city and 
burned trust documents belonging to others. 

From worry and self-accusation he passed into depression 
and agitation. He developed a belief that not only he but 
also his German wife were to be executed. He thought he 
was a criminal and was to be hanged. 

The depression then altered to a condition of hilarity and 
loquacity. 

In addition to the fixed pupils and absent knee-jerks, a 
speech disorder shortly developed. 

The patient was placed under care, but quickly (a few 
months?) passed into an advanced stage of paretic neuro- 
syphilis and died. 



NEUROSYPHILIS AND THE WAR 42 1 



SHELL-SHOCK PSEUD OPARESIS (non-syphUi- 
tic). Recovery. Case from Pitres and Marchand 
of Bordeaux. 



Case M. June 19, 1915, a shell exploded some distance 
from Lieutenant R. He remembers the gaseous smell, the 
bursting of several shells nearby and a sensation of being 
lifted into the air. When he recovered consciousness, he was 
in hospital at Paris- Plage, covered with bruises and scratches. 
They told him he had been delirious and had vomited and 
spat blood. 

June 24, his wife came to see him, but this visit he could 
not remember. Nor could his wife at first recognize him, he 
was so thin. He roused a few moments and recognized his 
wife, but relapsed into torpor again. Speech was difficult 
and ideas confused. 

A few days later he was able to rise ; but his mental status 
grew worse, especially as to speech and writing; the latter quite 
illegible. There was insomnia, or, if he slept, war dreams. 

August 7, he began a period of five months' convalescence 
passed with his family, depressed, given to spells of weeping, 
confined to bed or couch, unable to "find words," conscious 
of his state and troubled about it, speaking of nothing but 
the war, and afraid to go out for fear of ambuscade. There 
was at first a slight lameness of the right leg. Although he 
could walk, he felt pain in the knee on flexing the right leg 
on the thigh. He walked holding this leg in extension. 

On going back to the colors, he was immediately evacuated 
to the Centre Neurologique at Bordeaux, January 20, 1916. 

Examination found a bored, impatient, irritated man, vexed 
that a man who was not sick should be sent up ^'comme fou.'' 

Omitting negative details, neurological examination showed 
slight lameness as above, body stiff and movements jerky; 
difficult, unsteady gait. The lieutenant could stand for some 
time on either leg, tongue and face tremulous during speech. 
Limbs moderately tremulous, especially in the performance 
of test movements. 



422 NEUROSYPHILIS AND THE WAR 

Knee-jerks and Achilles jerks absent. Other reflexes, in- 
cluding pupillary, normal. Segmentary hypalgesia of right 
leg, especially about knee. Tremulous speech and writing. 
Patient would stop short in speaking for lack of words. 

Malnutrition. Appetite good, but a bursting feeling after 
meals. 

Skin dry, scaly on legs, fissured on fingers. 

Serum W. R. negative. Fluid not examined. 

Mental examination. Conscious and complaining of his 
troubles, Lieutenant R. claimed persistently that he was not 
sick. Memory for recent events was in general poor. Er- 
rands easily forgotten. Lost in the street. Complaint of 
corpse odors round him. Everybody is looking at him and 
making fun of him. He was apt to insult bystanders. He 
was afraid of German spies. Things in shops angered him 
as they seemed to him to be of German manufacture. 

There were frequent periods of depression, with pallor and 
no spontaneous speech for some hours to a half-day. Head- 
aches coming on and stopping suddenly. 

As to diagnosis, the first impression, say Pitres and Mar- 
chand, was that of general paresis. The progress of symp- 
toms after the shock was consistent with this diagnosis. The 
mental state and the physical findings seemed consistent, 
although the pupils were normal. His partial insight into 
his symptoms was not inconsistent with the diagnosis. He 
had a characteristic self-confidence. There had been four 
stillbirths (two twins); two children are alive, ii and 13. 
Typhoid fever at 30. Syphilis denied. No mental disease 
in the family. 

The patient had never done military duty, having been 
invalided for "right apex." But he had volunteered and been 
accepted in September, 19 14. 

I. Was this diagnosis, general paresis, at any time justified? 
The spinal fluid should of course have been examined. 
The peculiar lameness of the right leg was certainly 
not characteristic of general paresis, and was perhaps 
hysterical. (There was no limitation of visual fields or 
any other definite sign of hysteria.) Presumably some 
quality of speech defect, the amnesia, and the euphoria, 



NEUROSYPHILIS AND THE WAR 423 

together with absent knee-jerks, led to the diagnosis 
general paresis. By the 20th of March, 1916, the knee- 
jerks had become lively; the Achilles jerks normal. At 
this time the patient had gained In weight, could walk 
though stiffly, had headache (especially right frontal) 
and a feeling of lead In head, less tremor, lack of desire 
to undertake anything. He still wanted to go back 
into service. He still saw spies about. Dreams ter- 
rible; devoured by spiders, legglns instruments of 
torture. Skin still atrophic. June 4 there was no more 
tremor of speech or face. Symptoms largely disap- 
peared except a few ideas of persecution. Recovery 
October, 191 6. 
How was Lieutenant R. cured? Apparently by rest in 
the Centre Neurologique. PItres and Marchand do not 
speak of the subtle relation between mental state and 
the idea of non-return to military service. This motive 
might still work even if Lieutenant R. kept protesting 
quite sincerely that he wanted to go back into military 
service. 



424 NEUROSYPHILIS AND THE WAR 



SHELL-SHOCK PSEUDOTABES (non-syphiUtic, 
senim W. R. positive). Improvement. Case from 
Pities and Marchand of Bordeaux. 



Case N. Innkeeper B., 36, a shell-shock and burial victim 
June 20, 191 5, was looked on by a number of physicians as 
a case of genuine tabes. 

Even eight months after the episode, he still showed (when 
observed by Pitres and Marchand, February 3, 191 6) 
absence of knee-jerks and Achilles jerks, a slight swaying in 
the Romberg position, pupils sluggish to light, incoordination, 
delayed sensations. There was also a history of pains in the 
legs, compared by the patient to those of sciatica. These 
pains came in crises, the longest of which had lasted 30 hours. 

It seems that this soldier's troubles began the day after his 
shock with a feeling of swollen feet and of cotton wool under 
them. He stayed on service, however, walking with in- 
creasing difficulty. 

At the time of his evacuation, July 10, he could walk with 
great difficulty. "Strips of lead were between his legs." He 
could hardly control movements in the dark, or descend 
stairs. Often his legs would bend under him. Vesical func- 
tion sluggish. 

After a few months the patient could walk better. On 
February, 19 16, he walked thrusting his legs forward trem- 
bling, and dragging toes a little. He could not support him- 
self on either leg. Jerkiness and incoordination in extension 
or flexion of leg on thigh. 

The muscular weakness was decidedly against tabes or at 
all events a pure tabes. The incoordination proved to be due, 
not to loss of position sense (which was intact) but to un- 
steady muscular contractions. Deep sensibility was intact. 

There were no mental symptoms. There was a slight 
hesitation in speech and doubling of syllables, but nothing 
demonstrable with test phrases. 

The serum W. R. was positive. Syphilis denied. 



NEUROSYPHILIS AND THE WAR 425 

What is the cause of these phenomena? Pitres and 
Marchand lean to the hypothesis of sHght internal 
traumatism. They believe that there is either (a) slight 
internal hemorrhage in the nervous system, or possibly 

(b) what they call "nerve cell contusion," or perhaps 

(c) caisson-disease-like phenomena from aerial decom- 
pression. Some authors incriminate (d) the gases. It 
has been reported by certain French authors that 
shortly after shell shock injury or burial there is a 
pleocytosis in the spinal fluid as well as evidence of 
hemorrhage. The pleocytosis is said to last only a short 
time; hence when patient arrives at a base hospital 
lumbar puncture usually discloses nothing. 



Baalim and Ashtaroth 

Paradise Lost, Book I, line 422. 



VII. SUMMARY AND KEY 

No more important human problem now exists than 
syphilis. Syphilis of the nervous system or, briefly, neuro- 
syphilis is a highly important fraction of the total problem. 
The few outstanding dates and items which we present on the 
following page give but a faint idea of the amount of observa- 
tion and thinking which the medical aspects of neurosyphilis 
alone have required. The present work deals with but a small 
fraction of the results of this work, nor can we more than 
glance at the scientific history of syphilis and neurosyphilis 

— a history that would form an epoch in itself. 

It is only in the most recent years that syphilology and 
the narrower science of neurosyphilology have threatened to 
become separate disciplines boasting full time specialized 
workers. Up to recent years the contributions to the theory 
of syphilis have been largely by-products of work in larger 
sciences and arts. Thus, the cellular pathology of syphilis 
as worked out by Virchow and the more special vascular 
features as worked out by Heubner were incidental in the 
progress of pathological anatomy and histology. The bold 
procedure of Quincke in proposing lumbar puncture also had 
its more general ground in the extension of clinical medicine, 

— an interpretation likewise true of the French achievements 
in the cyto-diagnosis and chemical diagnosis of the lumbar 
puncture fluids. The careful histological definitions of the 
Nissl-Alzheimer group were incidental to the application of 
approved and classical pathological methods to neurological 
and psychiatric material. 

Again, the work of Schaudinn, as well as that of Metchnikoff 
and Roux, was ingenious work with the methods of para- 
sitology and experimental pathology. The great work of 
Schaudinn in establishing the constancy of the spirocheta 
pallida in syphilis may be said to have started syphilology 
as something approaching a special discipline. The ideas 
of one of the greatest of immunologists, Bordet, were almost 

427 



428 



SUMMARY AND KEY 



DATES, NEUROSYPHILIS 




VIRCHOW 


PATHOLOGY 


1858 


HEUBNER 


ENDARTERITIS 


1874 


QUINCKE 


LUMBAR PUNCTURE 


1891 


RAVAUT, SICARD, 


CYTODIAGNOSIS, 


1901 


NAGEOTTI, WIDAL 


C.S.F. 




WIDAL, SICARD, RAVAUT 


ALBUMIN, C.S.F. 


1903 


METCHNIKOFF AND 


TRANSMISSION TO 


1903 


ROUX 


APES 




ALZHEIMER 


HISTOPATHOLOGY, 
BRAIN SYPHILIS 


1904 


SCHAUDINN AND 


SPIROCHETA PAL- 


1905 


HOFFMANN 


LIDA 




WASSERMANN, NEISSER 


SERUM DIAGNOSIS 


1906 


AND BRUCK 






PLAUT 


WASSERMANN REAC- 
TION, C.S.F. 


1908 


EHRLICH 


SALVARSAN 


1909 


SWIFT AND ELLIS 


SALVARSANIZED 

SERUM 


1912 


NOGUCHI AND MOORE 


SPIROCHETES, BRAIN 
TISSUE, PARESIS 


1913 


LANGE 


GOLD SOL TEST 


1913 




Chart 28 



SUMMARY AND KEY 429 

immediately applied to the serum diagnosis of syphilis by 
Wassermann and the further application of this method to 
the problems of neurosyphilis was almost immediate, with the 
spirocheta pallida as an object of attack. The commanding 
intelligence of Ehrlich could at once seek application of long 
incubated ideas of chemotherapy with the startling outcome, 
salvarsan. 

The history of syphilis and neurosyphilis was now to be 
thickly sown with ideas and results growing from the achieve- 
ments of Schaudinn and Ehrlich. The positive reactions 
in the blood and spinal fluid in the most striking of mental 
diseases, general paresis, led to the impression that general 
paresis itself might at last be proved to be what Moebius had 
suspected, namely, 100% syphilitic. We know how difficult 
is the technical proof of spirochetosis in the brains of general 
paretics both post mortem and ante mortem, but no one 
doubts the certainty of the syphilitic hypothesis concerning 
the origin of general paresis. 

The data of the gold sol reaction ultimately obtained from 
the ideas of Thomas Graham concerning colloids, as developed 
by Szigmondi and effectively applied by Lange, have broad- 
ened and solidified the whole plane of attack. 

The ingenious suggestions of Swift and Ellis (salvarsanized 
serum) and the notable work of Noguchi and Moore (spiro- 
chetosis in paretic brains) indicate to us as Americans what 
the establishment of scientific institutes may do to permit the 
rapid application of new ideas to branches of inquiry that are 
opened out. Scientific institutes do not manufacture a 
Virchow, a Metchnikoff, a Schaudinn, a Bordet or an Ehrlich 
but they directly permit such men to work and indirectly 
stimulate the development of more. 

The series of 137 cases here at least presented does not 
touch systematically the problems of the neuropathology of 
syphilis, which would themselves require a textbook of respect- 
able size. We have, however, presented in Part I, cases i to 8, 
some indication of the protean nature of the material and 
from time to time in the remainder of the book somewhat 
fuller accounts of the pathological anatomy and histology 
have been presented than are strictly necessary in the .dem- 



430 SUMMARY AND KEY 

onstration of the principles of modern systematic diagnosis 
and treatment. 

Our work may be said to represent psychopathic hospital 
practice as available to us in our official capacities at the 
Psychopathic Department of the Boston State Hospital. 
A word is necessary concerning the nature of this practice. 
The dispensary and ward practice of a modern state psycho- 
pathic hospital, such as the Boston institution (founded in 
19 1 2) and the Ann Arbor institution (founded in 1906), is 
to be sharply distinguished from asylum practice. Those 
who have not followed the evolution of the modern psycho- 
pathic hospital with the lowering of bars to the admission of 
patients and the extension of its benefits to a group of sick 
persons far removed from the medicolegal concept " insan- 
ity " may not soon grasp the general nature of psychopathic 
hospital material. Psychopathic hospital practice stands, 
in fact, almost midway between asylum practice in the classi- 
cal sense and private practice. This has come about through 
the great extension of the so-called voluntary relation under 
which hundreds of patients now resort to the beds and out- 
patient rooms of a psychopathic hospital, who would formerly 
have remained untreated or inadequately treated. More- 
over, the broadening of the concept of mental diseases as a 
whole has permitted in some parts of the world the establish- 
ment of laws under which psychopathic and psychotic patients 
may be brought to psychopathic hospitals and even to asylums 
under the easiest possible conditions and restrictions, omitting 
court procedure altogether. The operation of the voluntary 
and temporary care provisions of law has accordingly yielded 
us, in the Boston institution, a great group of cases formerly 
not at all accessible to hospital diagnosis and treatment. 
Needless to say, as always under such conditions, we have 
been able to show not merely that hospital diagnosis or treat- 
ment is of importance to a new group of cases, but also that 
home treatment, especially home treatment under super- 
vision, is possible and even ideal for a large group of cases 
about which utter darkness or profound misgivings ruled in 
the not very distant past. 

Accordingly, we are fain to insist that our material is of 



SUMMARY AND KEY 43 1 

importance in new programs of community organization for 
the stamping out of disease. The work in psychopathic 
hospitals upon neurosyphiHs in particular is essentially a 
part of the public health program, although our special work 
will not soon be taken over by the public health officers, 
so complicated are the ramifications of medical and social 
diagnosis and treatment in the neurosyphilis group. 

We have tried in Part IV (medicolegal and social cases) 
to give a few examples to illustrate the part played by neuro- 
syphilis in society; but we regard this part of our work as the 
least satisfactory and the least representative in the total 
work. Our colleagues in social service, in mental hygiene, 
in psychopathology and in criminology will easily in the next 
few years provide a far more adequate basis for a full account 
of the public and social aspects of neurosyphilis. One point 
we should emphasize here. The psychopathic hospital worker, 
whether physician or social worker, must shortly decide 
upon and consolidate a program with relation to the families 
of neurosyphilitics. 

The syphilographers of the dermatological and special 
syphilis clinics have their identical problems with the families 
of syphilitics; but the dispensaries for mental cases and in 
particular the psychopathic hospital and asylum out-patient 
departments tap another reservoir of syphilitic families at a 
stage when the memory of the initial horrors of syphilitic 
infection is dimmed or. erased. Any program for the diagnosis 
and treatment of syphilis of the innocent must take into 
account not only the skin, syphilis, and internal medicine 
clinics but also the clinics for mental and nervous diseases 
wherein neurosyphilitics are not infrequent. Whether the 
ultimate percentage will stand at lo, 15 or 20% for the neuro- 
syphilitics in mental clinics, is of no importance to the prin- 
ciple. There are enough neurosyphilitics having economical 
importance and humanly precious families to warrant definite 
steps. 

The Massachusetts Commission for Mental Diseases has 
in the last few years employed the services of two medical 
workers whose time has been largely devoted to the applica- 
tions of our recent knowledge in neurosyphilis and has gone 



432 SUMMARY AND KEY 

SO far as to establish a neurosyphilis ward in one of the district 
state institutions (Summer Street, Worcester, under the 
Grafton Hospital Board). Special social workers in the 
field of neurosyphilis have also been available from time to 
time. These social workers are enabled with the support 
of the medical profession to do a great deal of good, for ex- 
ample, with the slogan The Child of a Paretic is the 
Child of a Syphilitic. 

The nature of the intake of patients into psychopathic 
hospital wards and out-patient clinics is such that great 
numbers of non-mental syphilitics arrive for diagnosis and 
possible treatment. Moreover, the existence of syphilis in 
non-suspects is a fact picked up by the way in routine Was- 
sermann serum diagnosis. 

The mental clinic in the modern sense with the medico- 
legal bars lowered or well nigh removed, turns rapidly into 
a clinic for neurological cases as well. The German models 
for mental and nerve clinics are rapidly being imitated. 
The result of this administrative novelty in our hospital 
procedure has incidentally yielded us many representative 
cases of entirely non-psychotic and even non-psychopathic 
neurosyphilis. Our impression grows and deepens that the 
neurosyphilitic is seldom merely a spinal syphilitic. The 
neurosyphilitic is nearly always the victim not merely of 
spinal disease but also of intracranial disease. Per contra, 
the victim of intracranial neurosyphilis is almost always 
more or less importantly affected by spinal neurosyphilis. 

The net result of the modern work on neurosyphilis has 
been to bring the neurologist and the psychiatrist together 
upon one platform in diagnosis and more and more upon one 
platform in treatment. But aside from the clinical evidence 
that the neurosyphilitic is apt to be a victim of both brain 
syphilis and cord syphilis, the autopsy evidence is stronger 
still. Even the victim of tabetic neurosyphilis (" tabes 
dorsalis ") himself is rarely found at autopsy without more 
or less evidence of significant encephalic disease of a chronic 
inflammatory or degenerative nature. Aside from tabes 
dorsalis and Erb's paraplegia, the rule is almost universal 
that neurosyphilis is a matter of the entire nervous system. 



SUMMARY AND KEY 433 

In view of the generalization of neurosyphilitic process, 
one might question the advantage of any topical grouping of 
neurosyphilitic disease. Practically speaking, however, as we 
have shown in Chart 5, it seems advisable to separate the 
neurosyphilitic diseases into six roughly distinguishable groups. 
First, there is the great group that we have chosen to term 
diffuse neurosyphilis, including many of the cases of so-called 
cerebral or cerebrospinal syphilis of the neurological clinics 
and the group of cases that have been treated in private 
practice by internists and neurologists without recourse to 
institutions. These cases have lived at home and have not 
been socially hard to manage until the late phases of their 
disease when the victims, if poor, are sent to almshouses and 
infirmaries under municipal or state care. These are the cases 
which have been in the past regarded as most amenable to 
the classical iodid and mercurial treatment. Indeed there 
is record of numerous therapeutic successes in the group. 

Whereas the lesions in diffuse neurosyphilis are chiefly 
chronic inflammatory and degenerative changes of a diffuse 
nature (with vascular changes incidental or subordinate to 
the inflammation and the degeneration), there is an important 
and large group of cases that we have termed vascular neuro- 
syphilis in which the factors of inflammation and degeneration 
are subordinate to vascular insults. These are cases of syphi- 
litic arteriosclerosis and the best examples are victims of 
cerebral thrombosis. The clinical symptoms of the immediate 
attacks (of apoplectiform, epileptiform or other acute nature) 
are not in themselves distinguishable from the immediate 
effects of non-syphilitic vascular disease; nevertheless the 
establishment of their syphilitic etiology is of the utmost 
importance on account of the possibilities of treatment of 
the underlying syphilis. For, as the neuropathologist must 
always insist, the immediate effects of vascular insults whether 
syphilitic or non-syphilitic are much more extensive than the 
ultimate paralytic or residual irritative effects; and by con- 
sequence a greater optimism is justifiable in the confronting of 
these cases than the nihilistic observer is likely to entertain. 

Physicians dealing with chronic disease in general are apt 
to be somewhat nihilistic, but this nihilism is increased a 



434 SUMMARY AND KEY 

hundred fold in disease of the nervous system. How Im- 
portant then is any work which shall demonstrate partial 
or even complete recovery from serious looking apoplectic 
and other seizures, besides all of which the point of syphilitic 
treatment naturally lies in the prevention of future insults 
of the same sort. Therapeutic experience in this vascular 
group has almost as good a toll of successes as in the diffuse 
neurosyphilis group above mentioned, that is to say, the 
modern systematic treatment and even the old pre-salvarsan 
treatments have succeeded fairly well in removing the products 
of inflammation from the membranes of the nervous system 
and in abolishing vascular disease. 

The old principle that the dead neurone in the central 
nervous system cannot be regenerated remains a perfectly 
firm principle ; but there are any number of neurones and even 
neurone systems that are not essential to life or to the pursuit 
of happiness. We accordingly have just as good a theoreti- 
cal therapeutic outlook in many instances of chronic neuro- 
syphilis as we have in chronic diseases of many other organs. 
Add to this the fact that a great number of the most sharply 
defined and grave symptoms are probably not due to de- 
struction of neurones but to irritation and functional dis- 
ability of neurones, and the conclusion is compelled that, as 
hinted above, an entirely unjustifiable pessimism and nihilism 
have prevailed In some quarters. Of course, the recoil from 
such pessimism with the onset of salvarsan treatment led 
various enthusiasts to an undue optimism. 

Another great group distinguished by the existence of 
spinal cord disease Is the group we have termed tabetic 
neurosyphilis, which group contains the classical tabes dor- 
salis or locomotor ataxia and its congeners. 

The question of therapeutic optimism comes up most 
forcibly in the field of tabes. It is hard, however, at this 
time to give a proper and scientifically founded estimate of the 
therapeutic outcome In tabetic neurosyphilis with modern 
methods. So much can be said : namely, that the alleviation 
of pain and the palliation of other symptoms can be success- 
fully claimed as a result of the renewed interest In the treat- 
ment of this affection. What was said above concerning the 



SUMMARY AND KEY 435 

finality of the death process in a dead neurone is very strik- 
ingly true, of course, of some of the neurones of the posterior 
columns in tabes dorsalis. Still only portions of these neu- 
rones (namely, those which run an intradural course) are 
strikingly altered in a great many cases. Now and again 
one is greatly astonished to observe the restoration of the 
lost knee-jerk in cases of neurosyphilis (see for instance the 
case of Alice Morton (i), with discussion). In short, the re- 
lation of several tabetic symptoms to irritative conditions 
and functional disability of neurones may be considered 
established. Naturally, moreover, if therapy can stop the 
upward course of the affection as it passes from lower to 
higher nerve roots (according to reasonably well-established 
ideas of the genesis and progress of this affection), we are 
entitled to a further degree of optimism. 

The question of therapeutic optimism versus pessimism is 
forced upon attention in the fourth great group of neurosyph- 
ilitic diseases which we have chosen to distinguish, namely, the 
group of paretic neurosyphilis including the disease formerly 
known as general paresis, paralytic dementia, softening of 
the brain and the like. 

Of course, no one can gainsay there is a group of cases 
having in the natural course of events a prognosis of fatality 
within a term of years, say three to five years, and we have 
cases in our series which go to show that even with the modern 
intensive treatment the characteristic down-grade sympto- 
matic progress and ultimate fatality occur. Still, we have 
other cases diagnostically on all fours with the fatal cases 
that have seemed to get either entirely well with the labora- 
tory tests returning to normal and without further mental 
symptoms, or else lose mental symptoms on the one hand or 
laboratory signs on the other. We should strongly object 
to any account of paretic neurosyphilis which should insist 
that its necessary outcome is fatality within a term of years. 
Of course, viewing our knowledge of the affection in the past, 
we should be compelled to object to the generalization " par- 
esis fataV on the evidences of the universally recognized re- 
missions. If nature can stop a paretic process, why cannot 
man do as much? Can it be alleged that our own apparent 



436 SUMMARY AND KEY 

therapeutic successes and those of others are merely curious 
examples of coincidences, namely, that remissions have chosen 
to occur precisely when therapy was systematically applied? 
The percentage of therapeutic successes with modern intensive 
treatment, wherever it may ultimately stand, is already too 
high for this hypothesis of fortuitous remissions.* 

Moreover, we believe that the details of the clinical progress 
of some of the reported cases are convincing on this point. 
What, however, is the distinguishing feature of paretic neuro- 
syphilis? It is in one sense a particular kind of diffuse neuro- 
syphilis. The tissues are apt to show not only encephalic but 
also spinal changes. There is apt to be a more or less well- 
defined meningitis, but the characteristic feature, without 
which the diagnosis of paretic neurosyphilis would hardly 
be rendered, is the existence of disease of the cerebral cortex. 
This disease is parenchymatous in the sense of showing nerve 
cell destruction. There is also an interstitial reaction in the 
shape of a neuroglia overgrowth, but the striking and pathog- 
nomonic feature is the infiltration of the sheaths of the 
small vessels in the cortex, giving evidence of an inflammation 
very intimately affecting the cellular mechanisms of the 
nervous system. It is striking how often a smaller or larger 
share of the cells found in the vessel sheaths are plasma cells. 
It does not appear, however, that the diagnosis of paretic 
neurosyphilis as against diffuse non-paretic neurosyphilis can 
be made in the stained sections with complete safety on the 
basis of plasmocytosis in the former and lymphocytosis in 
the latter. Whatever the results of careful histological differ- 
entiation by future neuropathologists may yield, it is at all 
events true that we cannot yet make an important differen- 
tiation clinically on the basis of the differential count of 
plasma cells and lymphocytes in the puncture fluids. How- 

* We have recently reviewed the outcome in 300 untreated 
cases of paretic neurosyphilis (Psychopathic Hospital ma- 
terial, strictly comparable with treated cases) finding but 5 
now capable of self-support and 10 more in normal-looking 
remission. This percentage is far lower than that in treated 
cases (at present, July, 1 917, 50 in 200 capable of self- 
support). 



SUMMARY AND KEY 437 

ever this may be, there is an important distinction between 
diffuse neurosyphilis of the non-paretic type and paretic neuro- 
syphilis in that paretic neurosyphilis rarely if ever fails to 
show important degrees of intracortical perivascular inflam- 
mation with larger or smaller numbers of plasma cells. 

What has the therapeutist to face in this matter? The 
answer, as elsewhere, depends somewhat upon what the future 
may decide as to the habitat and toxic or antitoxic activities 
of the spirocheta pallida. The early claims that the spi- 
rocheta pallida was extravascular and lay for the most part 
in the parenchyma and not in the vessel sheaths were perhaps 
overbold, since other workers have found the spirochete in 
the vessel sheaths also (Mott). 

Aside from the spirochete and its accessibility to spiro- 
chetocidal drugs, there seems to be no reason for supposing 
that the perivascular sheaths cannot be cleansed of their 
inflammatory contents. There is, again, no reason why the 
phagocytic cells should not continue to perform their scavenger 
function until such time as the degenerative process in the 
parenchyma (a process not necessarily progressive in the ab- 
sence of the spirochete or its products) ceases. There is every 
reason to suppose that a great many of the clinical phenomena 
are not necessarily due to permanent destruction of neurones 
and neuronic organs (dendrites, axis-cylinders, nets and the 
like) but are due to various microphysical conditions of 
pressure, intoxication and the like. 

The inflammatory conditions in the spinal cord of poliomye- 
litis, which conditions are precisely as striking as those of the 
paretic cortex, are beyond a question cleared away in the 
progress of the affection. Reference to the paradigm case (i) 
will show the type of our argument. There is no manner of 
doubt that in this paradigm case almost every portion of the 
nervous system had been sometime swept by spirochetosis 
and many of its small vessel sheaths stuffed with chronic 
inflammatory products. As for paretic neurosyphilis itself, 
a great many of its most striking clinical phenomena, such as 
loss of memory and disorientation, as well as great degrees of 
apparent dementia, are found virtually as often in cases with 
very slight anatomical changes as in cases with marked cortical 



438 SUMMARY AND KEY 

devastation. The inference is plain, that these phenomena 
are to a degree functional rather than structural. 

In brief, we conclude not only from therapeutic experience 
but also on a priori grounds that the histological conditions in 
paretic neurosyphilis are not entirely hopeless, and certainly 
not more hopeless than conditions in many chronic diseases 
outside the nervous system. Accordingly, we plead for a tem- 
perate optimism as to therapeutic results in general paresis. 

A fifth group of neurosyphilitic cases bulking rather largely 
in textbooks of pathology is the group of the gummata. 
For a variety of reasons (therapeutic and otherwise) the 
actual number of gummata of the nervous system available 
for clinical or even for anatomical study is much smaller than 
the books might lead one to infer. 

The sixth and last of the main groups of neurosyphilitic 
diseases is that of the juvenile forms, among which we find not 
only diffuse forms without a special and well-defined course, 
but also characteristic examples of paretic and tabetic neuro- 
syphilis. The distinction of a juvenile or congenital group 
of neurosyphilitics is, on theoretical grounds, perhaps hardly 
defensible. On practical grounds, however, the juvenile 
neurosyphilitics do form a group having special relations to 
feeblemindedness, epilepsy and the like. 

We must be clearly understood as to the rough, six-unit 
classification just given. It is practical merely. For com- 
parison we have given in other charts more expanded lists 
of the diagnostic entities in neurosyphilis among which that 
of Head and Fearnsides is of special interest, see Chart 2, 
page 2 1 . 

We shall now proceed to a brief analysis of the findings in 
our chosen series of 137 cases. We shall not reproduce the case 
headings of these cases, but expand their statements where 
necessary and tie them together so far as possible into a 
reasonable and systematic statement of the situation in neuro- 
syphilis. The footnotes will contain references to other cases 
in which identical points are illustrated as in the leading 
cases. The leading cases will in all instances be placed first 
in the footnotes. 



SUMMARY AND KEY 439 

The paradigm * shows meningeal, vascular and paren- 
chymatous lesions and thus illustrates our definition of the 
term Diffuse w^hich means precisely meningeal, vascular and 
parenchymatous. The meningeal lesions gave rise to two 
prominent sets of lesions, first, the marked tabetic lesions of 
the spinal cord (due to the spinal root neuritis incidental to 
the spinal meningeal inflammation), secondly, the character- 
istic asymmetrical and focal atrophy of cranial nerves inci- 
dental to a now largely extinct meningeal process at the base 
of the brain. The vascular lesions are responsible for another 
important and characteristic factor in the case, namely, the 
bilateral pyramidal tract sclerosis ; the bilateral cysts of soften- 
ing of the corpora striata are characteristic effects of old 
syphilitic cerebral thromboses. The parenchymatous disease 
in our paradigm is everywhere obvious, less so perhaps in 
the cortex itself than elsewhere, although here also evident 
in the shape of lesions suggesting an early phase of tissue 
atrophy. 

The paradigm Is of interest in demonstrating what in broad 
lines must be taken as an ascending disease proceeding not 
only from spinal cord to encephalon but also traceable as 
proceeding from lower parts of the spinal cord to upper parts 
thereof and from the lower encephalon to the higher structures 
of the cerebral cortex itself. 

The paradigm insistently calls attention to the advantage 
of persistent therapy not only in its display of remarkable 
successive recoveries from permanent looking symptoms but 
also histologically from the remnants of inflammatory process 
to be found in an otherwise almost wholly dismantled nervous 
system with extinct lesions. 

Tabetic Neurosyphilis f ("tabes dorsalis"), of course, 
often proceeds to death without special complications of syphi- 
litic nature. We have chosen a case, however, to demonstrate 
a terminal complication with vascular Insult. Incidentally the 
case shows another complication Inasmuch as the cause of 
death was rupture of aortic aneurysm. It is important to 
bear in mind these complications In tabes dorsalis which go 

* Alice Morton (i). f Francis Garfield (2). 



440 SUMMARY AND KEY 

to prove that the spirochetosis of tabetic neurosyphilis is not 
limited to the region of the spinal roots or to the spinal region 
in general. Tabetic neurosyphilis is apt to be only a part of a 
total picture of neurosyphilis just as neurosyphilis itself is 
only a part of the general syphilitic process. 

Our case of Paretic Neurosyphilis * ("general paresis") 
is a characteristic one in duration (three years and three 
months). The aortic sclerosis almost constantly found in 
neurosyphilis and especially in paretic neurosyphilis is here 
also shown. The spinal cord showed lesions which are also 
almost always found in paretic neurosyphilis. The character- 
istic frontal emphasis of the atrophic and indurative lesions is 
shown. There is also a display of gross changes in the pia 
mater. The characteristic so-called granular ependymitis or 
sanding of the ventricular surface is shown. The case is dis- 
tinguishable from the paradigm in not showing the effects of 
vascular insults in the shape of cysts of softening. The cere- 
bellar sclerosis of the case is fairly characteristic of paretic 
cases. There is even a suggestion of atrophy in the temporal 
region suggesting the so-called Lissauer's paresis. Clinically 
the case belongs in the classical grandiose group of paretics 
(" O. K. No. I superfine "). 

Vascular Neurosyphilis f is illustrated in a fourth autop- 
sied case. It may be noted that the pia mater in this case is 
practically normal. The tissues outside the area of softening 
due to the syphilitic thrombosis of nutrient vessels are practi- 
cally normal. The case was one of almost complete sensory 
aphasia with word-deafness. The clinical picture is accord- 
ingly quite distinct from those of the paradigm (i) and of the 
case of general paresis (3) just discussed. 

Juvenile Paresis J is illustrated by a case with exceedingly 
extensive lesions, largely meningeal and parenchymatous. 
The cerebral lesions are atypical since in places they suggest 
the tuberous sclerosis of Bourneville. The brain atrophy is 
extreme (965 grams) and it is possible that this apparent 
brain atrophy was in part hypoplasia, since the spirochetosis 

* John Dixon (3). f James Pierce (4). 

{ John Lawrence (5) , 



SUMMARY AND KEY 44 1 

of this case was doubtless congenital. However, clinically 
the patient was fairly normal up to the age of i8. 

A case of so-called Syphilitic Extraocular Palsy * de- 
monstrates a characteristic meningeal process more extensive 
than the clinical symptoms would have indicated. In fact, 
focal clinical nerve palsies are as a rule, if not constantly, 
partial phenomena of a far more extensive process of neuro- 
syphilis. They are far more limited clinically than anatom- 
ically and histologically. It seems at first sight improper 
to term them cases of diffuse neurosyphilis in view of their 
clinical focality, yet they are best described as partial cases of 
diffuse neurosyphilis. 

A case of Gumma f of the left Hemisphere is presented 
which appears to have led to death in about four years from on- 
set. This case, like many others, is not an example of purely 
focalized syphilitic process inasmuch as cysts of softening 
indicating slight vascular insults are present elsewhere (pons). 
There is also a degree of leptomeningitis, particularly basal. 

Our discussion of the nature and forms of neurosyphilis 
is completed by a rare case probably belonging in the so- 
called cervical hypertrophic meningitis of Charcot but actually 
due to a Gumma of the Spinal Meninges 4 The import- 
ance of therapeutic optimism is emphasized in this case as in 
the paradigm. Theoretically the meningeal inflammation of 
neurosyphilis ought to be almost entirely if not entirely 
removed by therapy, and these two cases, like several others 
in the series, seem to illustrate this possibility. 

Neurosyphilis sometimes receives the clinical diagnosis neu- 
rasthenia simply through omission to apply proved diagnostic 
methods. An instance is given in which the Paretic form 
of Neurosyphilis (" general paresis ") received the diagnosis 
neurasthenia § for a period of five years, at any time during 
which period it would doubtless have been possible to render 
the correct diagnosis and apply treatment. 

* Flora Black (6). § Greeley Harrison (9). Also 

j.i\/r T 4. /*,\ Albert Robinson (45), 

t Mrs. Lecompte (7). Alice Caperson (46), 

% John Wyman (8). Abel Bachmann (74). 



442 SUMMARY AND KEY 

Neurosyphilis may imitate not only the psychoneuroses 
but also the psychoses themselves. We present a case of an 
architect, which looked almost precisely like manic-depressive 
psychosis * and had a history of attacks, but in which the 
positive serum W. R. led (in accordance with hospital rules) 
to an examination of the spinal fluid. The spinal fluid tests 
proved the case to be one of Paretic Neurosyphilis. 

However, a positive serum W. R., even when associated 
with mental symptoms, and when those mental symptoms 
include grandiosity, does not prove the existence of neuro- 
syphilis either in its paretic or non-paretic form. Our in- 
stance seems to be one of Manic-Depressive Psychosis, f 
The spinal fluid tests were entirely negative. The course of 
the disease was also that of manic-depressive psychosis. In 
the absence of positive spinal fluid tests, the diagnosis neuro- 
syphilis was excluded. 

Neurosyphilis and even Paretic Neurosyphilis may 
result in symptoms that would ordinarily lead to the diag- 
nosis dementia praecox.X 

It is important not to rule out neurosyphilis on the ground 
of a negative serum W. R. The fluid W. R. may turn out 
positive. We present a case (of a salesman)! in which the 
serum W. R. was repeatedly negative (even salvarsan did 
not .act provocatively) yet the spinal fluid W. R. proved 
positive. The case was clinically one of classical Paretic 
Neurosyphilis ("general paresis"). It is a good rule to pro- 
ceed to lumbar puncture, even when the serum W. R. is nega- 
tive, if there are suspicious symptoms (e.g., speech defect and 



* Lyman Agnew (lo). Also | Henry Philipps (12). Also 
Ethel Hunter (47), Bridget Curley (59), 

Bessie Vogel (52), Margaret O'Brien (68), 

Isaac Thompson (83), Annie Martin (117). 

Juliette Lachine (11). 

§ William Twist (13). Also 

t JulietteLachine(ii)..4Z50 Lester Crane (20), 

Lyman Agnew (10), Thomas Donovan (23). 

Ethel Hunter (47), 
Bessie Vogel (52), 
Isaac Thompson (83). 



SUMMARY AND KEY 443 

memory impairment, grandiosity) or signs (e.g., marked reflex 
disorder, especially pupillary disorder). 

Diffuse Neurosyphilis was above defined as "meningo- 
vasculoparenchymatous. ' ' This disease is typically associated 
with six positive tests (positive serum W. R., positive fluid 
W. R., pleocytosis, gold sol reaction, positive globulin re- 
action and excess albumin). One or more and frequently 
several of these six tests are likely to run mild in diffuse 
neurosyphilis; that is to say, these tests are apt to run milder 
than the identical tests in paretic neurosyphilis ("general 
paresis"). The clinical course of the diffuse, and especially 
the meningovascular cases, is likely to be protracted. The 
prognosis as to life is good, barring fatal vascular insults. 
The illustrative case * was a case with slow course. There 
was a series of attacks followed by a paralytic stroke, a find- 
ing highly typical of the diffuse form of neurosyphilis. The 
spinal fluid reactions were mild, suitable to the general prin- 
ciple above stated. 

These tests are likely to run stronger, as above stated, 
in paretic neurosyphilis (" general paresis "), than in the 
diffuse form. In particular, the gold sol reaction is likely 
to be shown in what is termed " paretic " form rather than 
in what is termed " syphilitic " form. The clinical course of 
Paretic Neurosyphilis is likely to be brief. A character- 
istic case t with very heavy globulin and albumin tests is 
presented. 

Tabo-paretic Neurosyphilis f ("taboparesis") is clin- 
ically a combination of the symptoms of tabetic ("tabes 
dorsalis ") and those of paretic neurosyphilis (" general 
paresis "). First comes the tabes dorsalis lasting often 
for many years. Afterward follows a characteristic general 
paresis. The ultimate paretic picture is likely to retain, 
however, various characteristics of tabes. The laboratory 
tests in the paretic phase of taboparesis are characteristic 



* John Jackson (14). Also f Pietro Martiro (15). Also 
Martha Bartlett (21), Meyer Levenson (22), 

Paolo Marini (28), Achilles Akropovlos (50). 

Margaret O'Brien (68), % Joseph Sullivan (i6). 



444 SUMMARY AND KEY 

of general paresis and not of tabes dorsalis. The prognosis 
after the paretic phase has arrived is that of general paresis. 

The diagnosis of the neurosyphilitic forms would be easy 
if these principles were always carried out to the letter. 
The important fact is as follows: diffuse (that is, meningo- 
vasculoparenchymatous neurosyphilis) may look like paretic 
neurosyphilis (" general paresis ") * at certain periods of the 
clinical and laboratory examination. This fact is of obvious 
importance. The general prognosis of diffuse neurosyphilis 
is regarded as good quoad vitam. The general prognosis of 
paresis is bad. If, however, the differential diagnosis cannot 
be rendered at particular phases of a given case, then no safe 
prognosis can be offered in the individual case. In particu- 
lar no prognosis affecting the administration or non-adminis- 
tration of modern systematic treatment can or should be 
offered in these doubtful phases. 

It is not always safe to exclude neurosyphilis even when the 
fluid W. R. is negative, f Particularly in vascular neurosyphi- 
lis the fluid W. R. and even all the other laboratory signs 
in the spinal fluid may sometimes be negative. A positive 
serum W. R. yields the correct pointer to diagnosis. Of 
course, also in many cases of vascular neurosyphilis one or 
more of the laboratory signs may be suggestive even when 
the fluid W. R. is negative. Theoretically there may be 
cases in which all the six tests are negative and yet the diag- 
nosis neurosyphilis be the correct one. 

A clinically important sign in neurosyphilis is the so-called 
seizures. These occur both in Diffuse Non-paretic Neuro- 
syphilis % and in Paretic Neurosyphilis. § 



* Gregorian Petrof ski (17). Also | Agnes O' Neil (19). Also 

Richard Lawlor (25), Michael O'Donnell (24). 

John Bennett (34), John Edwards (104). 

Julius Kantor (54), Arthur Bright (121). 

Albert Forest (112). 

§ Lester Crane (20). Also 

t Frederick Wescott (18). Also Greeley Harrison (9). 

Martha Bartlett (21), David BorofskI (49). 

James Burns (56), David Collins (61). 

Victor Friedburg (108). 



SUMMARY AND KEY 445 

Aphasia is likewise a symptom in both these forms of 
neurosyphilis, namely, in the Diffuse non-paretic * and in 
the Paretic form.f 

The literature contains reference not only to seizures and 
aphasia as characteristically paretic but also to remissions. 
Remissions like seizures and aphasia are found in both the 
Paretic | and Non-Paretic forms of neurosyphilis. § They 
have important bearings on prognosis in all forms of neuro- 
syphilis and are of especial significance in the evaluation of 
treatment. (Remissions coincident with apparent cure.) 

So far we have been dealing with cases of neurosyphilis 
in which there was no doubt of the existence of mental 
symptoms. There are cases, however, in which although the 
laboratory signs of neurosyphilis exist, proving beyond doubt 
the existence of a chronic inflammatory reaction and allied 
pathological conditions in the cerebrospinal axis, there are no 
mental symptoms of neurosyphilis. We have called some of 
these cases Paresis Sine Paresi || and present examples. 

To illustrate complications we give a case of Paretic Neu- 
rosyphilis with autopsy in which there were ante mortem 
signs of Herpes Zoster ^ or, at all events, of a skin eruption 
limited to the area of a thoracic nerve. 

A case of Gumma of the brain ** in which decompression 
was warranted and performed is presented. The fluid W. R., 
as in many such cases, was negative; serum positive. 

A case of Cranial Neurosyphilis (extraocular palsy ft 
without mental symptoms) showed a positive Wassermann 
serum test and a negative spinal fluid. 

* Martha Bartlett (21). Also \\ Richard Lawlor (25). Also 
Agnes O'Neil (19), Bessie Vogel (52), 
Vivian Walker (87). (88). 

t Meyer Levenson (22). Also ^ John Morrill (26). 

Albert Forest (112). ** d^^j^ Tannenbaum (27). 

X Thomas Donovan (23). Also Also Mrs. LeCompte (7), 

William Twist (13), Annie Rivers (109). 

Bessie Vogel (52), || p^olo Marini (28). Also 

David Collms (61). ^{0x2. Black (6). 

§ Michael O'Donnell {2^). Also 

Alice Morton (i). 



446 SUMMARY AND KEY 

The laboratory reactions in Tabetic Neurosyphilis * 
(" tabes dorsalis ") run somewhat Uke those of diffuse non- 
paretic neurosyphilis and are accordingly milder than those 
of paretic neurosyphilis. The fluid W. R. and the gold sol 
reaction in particular are apt to run mild. The clinical course 
of tabes dorsalis is well known to be protracted and the prog- 
nosis quoad vitam is good except that we must alway bear in 
mind the possibility of vascular insults and complications 
of a syphilitic origin in the rest of the body. 

It is important to remember that Tabetic Neurosyphilis 
is often quite atypical f clinically and may even show no 
single symptom warranting the old clinical name locomotor 
ataxia. 

There are even cases in which the name tabes dorsalis is 
not warranted in view of the fact that the lesions are not 
low in the cord but are higher up (Tabes Cervicalis|). 

A rare form of neurosyphilis is Erb's Syphilitic Spastic 
Paraplegia § against which one needs to consider a number 
of non-syphilitic spinal cord diseases. Our case showed a 
weakly positive serum W. R., a negative fluid W. R., and the 
other tests of the spinal fluid were moderately positive. 

Syphilitic Muscular Atrophy || is classified by Head and 
Fearnsldes both in their meningovascular group and in their 
group of the so-called syphilis centralis. Our case affecting 
in large part the small muscles of the hands In a teamster, 
may be due either to spinal parenchymal lesions or to root 
neuritis or to both. 

It is a little extraordinary and very important that the 
laboratory signs are apt to be positive even in the Secondary 
period of Syphilis. Perhaps a third of all cases of syphilis in 
the secondaries would, If tested, yield positives precisely 
like those of full-blown paretic or diffuse neurosyphilis. 

* Mario Sanzi (29). Also f Stephen Green (30). Also 
Stephen Green (30), Paul Halleck (31), 

Paul Halleck (31). Henri Lepere (105), 

Ivan Rokicki (in). 
% Paul Halleck (31). 
§ Margaret Neal (32). 
II Joseph Graham (33). 



SUMMARY AND KEY 447 

Strangely enough, these signs may occur without clinical 
symptoms. The illustrative case,* a mechanic, yielded vari- 
ous mental symptoms. The cases of secondary syphilis with 
laboratory signs of neurosyphilis but without clinical symp- 
toms are of the greatest theoretical importance in relation 
to the problem above mentioned of paresis sine paresi. It 
may well be inquired whether in some instances the neuro- 
syphilis of the secondaries does not persist until the ex- 
hibition of mental or physical symptoms of neurosyphilis 
years later. It must be remembered that this conception is 
hardly more than a hypothesis at the present time. That 
such signs of chronic inflammation could exist without 
symptoms is not so surprising when one thinks of the startling 
immediate improvement seen after treatment or even in 
remissions without treatment. One is reminded of the crisis 
in pneumonia wherein clinical improvement takes place en- 
tirely independent of the mechanical conditions in the lung 
which just after the crisis remain as suppurative as before. 

The diagnosis of Juvenile Neurosyphilis is made upon 
the same lines as that of neurosyphilis in the adult. We pre- 
sent two cases, one with optic atrophyf and the other with signs 
of congenital syphilis antedating the symptoms of paresis. J 

Congenital syphilis is also apparently capable of producing 
a simple form of Feeblemindedness, § that is to say, a form 
of disease non-paretic, non-tabetic, and without special tend- 
ency to vascular insults. 

We present a case of Juvenile Tabetic Neurosyphilis 
(" juvenile tabes ").|| The tests were all positive. 

The line of separation between typical and atypical cases 
of neurosyphilis is vague and indistinct and some of the 

* John Bennett (34). Also J Theresa Mullen (36). Also 
Alice Caperson (46), John Lawrence (5), 

Florence Fitzgerald (81), John Friedreich {77), 

Vivian Walker (87), Gridley Ringer (78), 

Arthur Bright (121). James Arnold (80). 

t Mary Coughlln (35). § Isaac Goldstein (37). 

II Archibald Sherry (38). 



448 SUMMARY AND KEY 

cases classified by us amongst puzzles perhaps belong under 
systematic diagnosis and vice versa. The section on PUZ- 
ZLES AND ERRORS in the diagnosis of neurosyphilis is 
introduced by six cases of error in the diagnosis of the paretic 
form of neurosyphilis.* These errors were made known by 
autopsy. Aside from the sixth case, whose etiology must re- 
main in doubt and which was a unique case of Perivascular 
Gliosis, there is ground for the belief that the other five cases 
in this Danvers Hospital study of diagnostic errors were per- 
haps actually syphilitic though not of the paretic form of 
neurosyphilis. At all events, the brain tissues in these cases 
failed to show the plasma cell deposits which are characteristic 
in the sheaths of the intracortical vessels in paretic neuro- 
syphilis. 

A case illustrates the complication of Tabes by arterio- 
sclerotic symptoms, in which case the arteriosclerosis may 
naturally have been of syphilitic origin. Two cases especially 
illustrate the possibility of confusing the ataxia of general 
paresis with Cerebellar Ataxia. These cases showed 
lesions of the cerebellar structures, notably of the dentate 
nucleus. No one can read these cases or any of the autop- 
sied cases in our series, without perceiving how fundamental 
and even critical is the demand for autopsies in fatal cases 
of neurosyphilis. The practitioner who can secure an autopsy 
in a fatal case of neurosyphilis and have the tissues worked 
up by approved neuropathologlcal methods is almost bound 
to add his bit to neurological theory. Even cases of classical 
tabes dorsalis are often signally important to the theorist on 
account of the relations of the neural to the non-neural com- 
plications. 

We then proceed to a group of cases without special order 
in which a variety of diagnostic questions arose. 

A case of questionable neurosyphilis In the secondary stage 
of syphilis brings up the problems of syphilitic neurasthenia.^ 

* Caroline Davis (39). Elizabeth Brown (42). 

H. F. (40). Robert Allen (43). 

Samuel North (41). John Hughes (44). 

t Albert Robinson (45). Also 
Greeley Harrison (9). 



SUMMARY AND KEY 449 

Syphilis may act as agent provocateur of Hysteria as 
Charcot insisted.* 

A case illustrative of difficulties in diagnosis between neuro- 
syphilis and manic-depressive psychosis follows.f 

A case for diagnosis is given which shows that errors in 
the diagnosis of neurosyphilis are entirely possible even when 
abundant clinical and laboratory data are available. A case 
with a weakly positive Wassermann reaction in the spinal 
fluid finally turned out to be one of Brain Tumor.J 

Some questions as to the diagnosis of Neurosyphilis versus 
Idiopathic Epilepsy are brought up by a] case in which phe- 
nomena of paresis seemed to have occurred very early, about 
two years after the initial syphilitic infection. § 

A case of Paretic Neurosyphilis is offered in which 
hemiplegia and hemitremor strongly suggested vascular lesions; 
but the autopsy showed no coarse lesions and merely con- 
firmed the diagnosis paresis microscopically. || 

An autopsied case of Paretic Neurosyphilis is given, in 
which the pupils persisted in reacting normally. Herpes 
zoster-like lesions in life yielded no special signs at autopsy 
(all root-ganglia looked alike above and below zone of 
"shingles.")^! 

An example of Neurosyphilis, probably Paretic, yielded 
symptoms highly suggestive -^of manic-depressive psychosis.'^* 
An interesting feature in this case was the birth of a healthy 
child nine months after the onset of the psychotic attack. 

An example of exophthalmic goitre ff following the ac- 
quisition of Syphilis showed at autopsy a heavy scarring of 
the optic thalamus and unilaterally atrophic process in the 
cerebral cortex. 

* Alice Caperson (46). Also § David Borosfski (49). Also 
Florence^Fitzgerald (81). Lester Crane (20). 

t Ethel Hunter (47). Also \\ Achilles Akropovlos (50). 

Lyman Agnew (10), \ Daniel Wheelwright (51). 

Bessie Vogel (52) ** .Bessie Vogel (52). Also 

Juliette Lachme (11). ^yman Agnew (10), 

I Milton Safsky (48). Also Juliette Lachine (11), 

Daniel Falvey (55). Ethel Hunter (47). 

tt Carrie Pearson (53). 



450 SUMMARY AND KEY 

We come to some questions concerning the Argyll-Robert- 
son pupil. It is agreed on all hands that the Argyll- Robert- 
son pupil is characteristic of the paretic and tabetic forms, 
but the sign occurs also in other neurosyphilitic conditions;* 
in fact the sign does not necessarily indicate neurosyphilis 
as an instance of Pineal Tumor demonstrates. f 

The question raised above as to the possibility that neuro- 
syphilis may exist in the absence of positive findings in the 
spinal fluid is illustrated in a man, a mechanic, who claimed 
syphilitic infection and showed an Argyll-Robertson pupil 
on one side.J The serum W. R. was positive; the fluid tests 
were negative. 

An extraordinary case is given in some detail in which 
Neurosyphilis in the form termed Disseminated Enceph- 
alitis! proved fatal within seven months of the initial in- 
fection. 

We have frequently mentioned the classical assumption 
that paretic neurosyphilis (" general paresis ") is a fatal disease. 
Some have suggested that there is another form clinically 
almost identical with general paresis except that it pursues 
a long course and the suggestion has been made that these 
cases be termed pseudoparesis .\\ We are of the opinion that 
this term should be dropped and advocate the use of the 
word pseudoparesis only for non-syphilitic disease looking 
like paresis, such as alcoholic pseudoparesis and the like. 

The question whether there is a form of mental disease 
Syphilitic Paranoia^ is raised by a case with auditory hal- 
lucinations, ideas of persecution and attacks of excitement. 
The diagnosis of alcoholic hallucinosis was actually made al- 
though there is no proof that the patient ever drank alcohol. 

Alcohol may cause symptoms identical with those of 

* Julius Kantor (54). Cf. % James Burns (56). Also 
James Burns (56). Frederick Wescott (18), 

Henri Lepere (105). Martha Bartlett (21), 

Frederick Stone (106). Victor Friedburg (108). 

t Daniel Falvey (55). Cf. § John Summers (57). 
Francis Murphy (60). || p^^^^ Burkhardt (58). 

^ Bridget Curley (59). 



SUMMARY AND KEY 45 1 

paretic neurosyphilis, including seizures, Argyll- Robertson 
pupils, speech defect and mental symptoms. The differen- 
tation is readily made by the negative laboratory findings. 
An illustration is given in our case of the alcoholic teamster. 
Cases such as this bear the name Alcoholic Pseudo- 
paresis.* 

However, when the clinical picture is the same as In the 
case of our teamster, the alcohol may only be a complicating 
factor in neurosyphilis, as shown by our next case of the 
alcoholic steamfitter who In fact was shown to have Neuro- 

SYPHILIS.f 

Sometimes cases of apparently frank alcoholism, even with 
apparently characteristic delirium tremens and neuritis, prove 
to be essentially neurosyphllltic.J On the other hand, true 
combinations of Alcoholism and Neurosyphilis occur which 
it would be proper to classify under either heading and in which 
therapy must take serious account of both conditions. § 

As above stated, we elect to use the term pseudoparesis only 
for non-syphilitic cases. There are other forms of pseudo- 
paresis than alcoholic pseudoparesis. The question of Dia- 
betic Pseudoparesis is raised by an exceedingly complicated 
case of which our best Interpretation is that the patient, a 
proved syphilitic (with syphilitic osteomyelitis (?)), a huge 
doorkeeper, was perhaps suffering from an old Syphilitic 
scarring of the Pituitary body.] | Neither this case nor a 
second case, one of Paretic Neurosyphilis with glycosuria 
is actually entitled to the diagnosis diabetic pseudoparesis. 
The second case of paretic neurosyphilis with glycosuria 
brings up some unanswerable questions as to the pancreatic 
or basal meningitic or other origin for the glycosuria.^ 

Isolated symptoms are often presented by neurosyphilitics 
(e.g., hemianopsia) ;** but we tend to regard these cases as due 
to focal lesions that are merely part and parcel of Diffuse 
Lesions. 

* Francis Murphy (60). § Albert Fielding (63). 
t David Collins (61). || Calvin Hall (64). 

% Joseph Buck (62). ^ Donald Barrle (65). 

** Lawrence Washington (66) 



452 SUMMARY AND KEY 

A neurosyphilitic case (a steward) with the rather unusual 
complication (for our northern region) of severe Malaria 
producing cerebral thrombosis Is reported.* 

The diagnosis Dementia Praecox] was actually made in 
the case of a young school teacher in whom the laboratory 
findings proved conclusively that the condition was one of 
Neurosyphilis. The gold sol reaction in this case was mild. 
The chief lesion at autopsy was a fresh looking, gelatinous 
plal exudate over the spinal cord which turned out to contain 
an almost pure display of very numerous plasma cells. 

The question of Lues Maligna f is brought up in a rectifier 
of spirits in whom the characteristic tremendous destruction 
of tissue, toxemia and failure to react to antisyphilltic treat- 
ment were Illustrated. Moreover, this case had a trauma 
(cautery) to the tonsil, as in other cases of lues maligna. 

A case somewhat suggestive of hrain tumor, of neuro- 
syphilis and of multiple sclerosis^ turned out to be Multiple 
Sclerosis (the fluid showed a pleocytosis and a moderate 
amount of globulin with a paretic type of gold sol reaction). 

As a foil to this case that we regard as multiple sclerosis, 
we present a second case with nystagmus, optic atrophy and 
spasticity in which the suspicion of multiple sclerosis might 
well be raised but which the tests demonstrated to be 

NEUROSYPHILITIC. 1 1 

An even stranger imitation of well-defined non-syphilitic 
entities was presented by a case apparently of Huntington^ s 
chorea^ (except for absence of the hereditary taint) which 
case, however, proved to the surprise of all diagnosticians to 
be one of Neurosyphilis. 

Frequent errors of diagnosis must occur in the field of the 
senile psychoses. We present a case that would at first 
blush warrant the diagnosis of senile arteriosclerotic psychosis** 

* Joseph Temple (67). % Frank Mason (69). 

t Margaret O'Brien (68). Also § Annie Kelly (70). 
Henry Phillips (12). James Lauder (71). 

Bridget Curley (59). || James Lauder (71). 

Annie Martm (117). ^y^^ ^ / ^ 

Tl Margaret Green (72). 

** Marcus Chatterton (73). 



SUMMARY AND KEY 453 

in a sea captain of 75 years (wife dead 15 years before of 
general paresis) who turned out to be a characteristic case 
from the laboratory standpoint of Neurosyphilis. 

The Protean nature of the symptomatology of neurosyphilis 
is sufficiently established. Still, a case that might fit into text- 
books concerning Dissociation of Personality * is certainly 
a clinical oddity, as illustrated by a fugacious musician. 

A case with strong suspicions of neurosyphilis of tabetic 
type turned out to be more probably one of neural com- 
plications in Pernicious Anemia. f 

Neurosyphilis in Juveniles presents puzzling conditions. 

One case was marked clinically by attacks of excitement. % 
It is impossible to place this case among the main groups of 
juvenile neurosyphilis. 

Another case of Feeblemindedness,! also Neurosyphi- 
LiTic in origin, presented physical symptoms and labora- 
tory signs of paretic neurosyphilis; yet this case had been 
considered one of simple feeblemindedness. 

A case apparently of Juvenile Paretic Neurosyphilis in 
a 15 year old boy presented the rather unusual complication 
of shocks with quadriplegia,| | a vascular complication not 
usually expected in the paretic type of neurosyphilis in adults. 

Epileptic phenomenal! are rare as the effect of Juvenile 
Neurosyphilis, but occur as demonstrated in a case which 
slipshod methods of diagnosis might well have regarded as 
one of idiopathic epilepsy. 

A case of Juvenile Paretic Neurosyphilis with the 
complication of Addison's Disease ** is given (autopsy 
confirmation). 

The puzzle in diagnosis offered by syphilis in the secondary 
stage 1 1 is illustrated by a case which showed the characteristic 
Neurosyphilitic complications of the Secondary Stage of 

* Abel Bachman (74). ** James Arnold (80). 

t Mrs. Brown (75). ft Florence Fitzgerald (81). Also 
X James Seabrook (76). John Bennett (34), 

§ John Friedreich(77). Cf. ^]^'^^ ^^SZ^'"'^ l'^^) ' 
Isaac Goldstein 37)- Y^T" ^^^^' .^^7). 

II Gridley Ringer (78). ^^^^^^ ^^^^^^ ^'^'^' 

^ John Doran (79). 



454 SUMMARY AND KEY 

syphilis. This patient may well have been a moron at the out- 
set and exhibited some reactions (refusal to talk) explicable on 
the basis of feeblemindedness. She was a neurosyphilitic only 
in the sense of the neural complication that we find in the 
secondary stage of syphilis. As stated above, we do not yet 
know what the fate of these neural complications of secondary 
syphilis is to be. The frequency of this finding in secondary 
syphilis is probably too great to warrant the hypothesis that 
it must always go on to a chronic neurosyphilis; but we 
certainly are warranted in regarding these cases as potential 
chronic neurosyphilitics. 

A case of Taboparetic Neurosyphilis in which the heavy 
exudate characteristic of paresis became a soil for a growth 
of the typhoid bacillus is presented with autopsy.* This 
fatality with Typhoid Meningitis is merely a concrete ex- 
ample of the many complications which syphilitics and especi- 
ally neurosyphilitics have to sustain. 

The case series then goes on to illustrate, though quite 
inadequately, a variety of MEDICOLEGAL AND SOCIAL 
complications of neurosyphilis. It is well known that many 
social complications with grave moral, economic and even 
political difficulties occur. 

Our series starts with a "public character"! whose elo- 
quence and reformatory efforts led to a considerable noto- 
riety. The autopsy in this case showed singularly few lesions 
despite the fact that the case was microscopically one of 
wholly characteristic Paretic Neurosyphilis. The ques- 
tion might arise how far we are entitled to correlate the refor- 
matory efforts of this always eccentric character with syphilis. 
The man himself a physician, was aware of the doubt which his 
Argyll-Robertson pupils threw upon his medical situation. 
He explained them on the basis of an old smallpox! We are 
inclined to think that the whole of this man's life, from his 
giving up of medical practice to live as a kind of literary and 
political hack, was due to subtle changes of neurosyphilitic 

* Frederick Estabrook (82). 

t Maj. Isaac Thompson, M.D. (83). 



SUMMARY AND KEY 455 

origin. The fact that there was a certain deHnquent streak 
in the man is not inconsistent with this idea. Interestingly 
enough, a fall on the ice in the man's 6ist year actually 
started up the fatal process, a condition of affairs amply 
illustrated in cases of neurosyphilis, brought out by trauma 
that come to the attention of the Industrial Accident Board 
in connection with claims for compensation. 

A case of sudden grandiosity* illustrates an episode of 
NEUROSYPHiLiTic Origin. Such a person might well be re- 
garded by the lay newspaper reader as a crank or a grafter but 
the neurosyphilitic possibility should always be entertained 
in cases of this order. 

As against the social difficulties that look in the direction 
of the classical paretic grandeur, we present a case of apparent 
suicidal attempt by gas, which attempt was followed by a 
period of amnesia that, taking into account the laboratory 
findings, was probably NEUROSYPHiLiTicf 

Vistas of extraordinary interest are opened out by studies 
of the relation of neurosyphilis to delinquency. The case 
of the psychopathic reformer (Case 83) above mentioned was 
one in which the delinquency may possibly have been related to 
acquired syphilis. We present also a case of juvenile neuro- 
syphilis, a young man of reform school type % in which Juve- 
nile Paretic Neurosyphilis was established. This patient, 
in fact, deteriorated very rapidly to a condition of considerable 
dementia a few months after the diagnosis was established. 

A striking case of so-called Defective Delinquency is 
presented, an alcoholic prostitute of the reformatory group. § 
The Neurosyphilis in this case was a complication rather 
than an original factor in the delinquency. 

One case of Paresis Sine Paresi was that of an habitual 
criminal || and forger who, without showing mental or physical 
symptoms of neurosyphilis, yielded the laboratory signs of 
paretic neurosyphilis. Again, as in the case of the prostitute 

* Lester Smith (84). § Vivian Walker (87). 

t Annie Marks (85). || (88). Cf. 

+ T7 1 T 1- rar\ Richard Lawlor (25). 

% Frank Johnson (86). g^g^j^ Vogel (52). 



456 SUMMARY AND KEY 

just mentioned, the Criminality seems to have antedated 
the neurosyphilis and even to have been hereditary. 

By way of introducing the next group of Industrial Ac- 
cident Board cases, we present a case of Juvenile Paresis 
with initial Traum . 

The Industrial Board group is of note in that the signs of 
the traumatic formf of paretic neurosyphilis do not occur 
immediately upon the accident. Some time elapses in which 
the physical, chemical or parasitological changes have time 
to work themselves out in the injured tissues. Many hy- 
potheses may be raised as to the reason why a trauma lights 
up a syphilitic process. Of course, false claims J may be 
made for compensation by neurosyphilitics in whom the 
symptoms were already in existence before the accident and 
in whom they may not even be markedly exacerbated by the 
accident. The false claimants can probably not readily 
frame a story which the expert psychiatrist cannot discredit 
if he is allowed to perform laboratory tests and give the patient 
the benefit of thorough examination. However, some cases 
of established Paretic Neurosyphilis are perhaps truly 
subject to exacerbations^ of the clinical process and it may 
well be held that such exacerbations warrant partial com- 
pensation. 

The fact that a trauma may light up a syphilitic process 
is illustrated in a case that came to the Psychopathic Hospital, 
in which a Syphilitic Lesion developed in the skull at the 
Site of Skull Injury. || 

A case of Occupation-neurosis ^ that might be interpreted 
as a syphilitic neuritis is presented. The case is still in doubt 
as to its scientific evaluation. 

The workmen's compensation group of syphilitic cases is 
of extraordinary general interest since it indicates that 



Margaret Tennyson (89). f Joseph O'Hearn (90). 

Ky^S-li^ [%. t Levi Sussman (91). 

Theresa Mullen (36). § Joseph Larkin (92). 

Sey"Rtge?(7^^^ H ^^^^^^d Marshall (93). 

James Arnold (80). ^ David Fitzpatrick (94). 



SUMMARY AND KEY 457 

employers may well be on the lookout not to employ known 
syphilitics unless fortified by special insurance arrangements. 
Whether in future employers may desire to employ only W. R. 
negative workmen is one of the highly complicated questions 
re workmen's compensation and health insurance. 

But the problems of neurosyphilis are not merely medico- 
legal and broadly public or social. The most appealing dif- 
ficulties lodge within the bosom of the family. Now and 
then a case of Incompatibility of Temperament, perhaps 
complicated by alcoholism, occurs which tests prove to be 

NEUROSYPHILITIC* 

Special attention should be drawn to a certain Neuro- 
SYPHiLiTic Family f in which both parents and five children 
showed a variety of syphilitic diseases, including syphilis 
without apparent neural complications, paretic neurosyphilis, 
juvenile paresis, aortic aneurysm, achondroplasia and caries 
of the spine, and an as yet indefinite neurosis. There was a 
sixth child that died shortly after birth, as well as three still- 
born. 

One cannot conclude from the normal % look of a neuro- 
syphilitic's family that the normal looking members are not 
syphilitic, as illustrated by the family of our draughtsman. 

The most intricate social complications may arise. We 
present a case of a syphilitic man (a well-to-do merchant) 
who was apparently being goaded into a second marriage § 
because he was continually being charged with having caused 
his first wife's death. This he had actually done in a certain 
sense because his wife had died of general paresis, having 
contracted syphilis from him. 

In the fifth section on THERAPY, we have attempted to 
outline some of the principles and problems that arise in the 
treatment of neurosyphilis. Enough has probably been said 



* Joseph Wilson (95). % Walter Heinmas (97). 

t Becky Bornstein (96). § Mr. Jacobs (98). 
Walter Heinmas (97). 
Mr. Jacobs (98). 



458 SUMMARY AND KEY 

concerning the attitude of optimism or pessimistic nihilism 
that may be adopted toward the whole subject. It must be 
borne in mind, however, that a great deal of the work on 
treatment of neurosyphilis is still in the experimental stage. 
As a rule, each case must be considered separately and in- 
dividually and the prognosis can be made satisfactorily only 
after treatment has been given. This section contains a 
group of cases that have been treated rather intensively and 
the results of this treatment are indicated. The section is 
introduced by five untreated cases, the brains and cords of 
which have been studied post mortem. These illustrate the 
pathological conditions which we have to meet, and from 
these examples we can draw the theoretical conclusion that 
some cases are beyond the aid of therapy on account of the 
brain destruction. Others, in which the symptomatology 
bespeaks just as grave a situation, turn out on autopsy to have 
very little actual damage to the brain tissues and therefore 
should theoretically at any rate be amenable to antisyphilitic 
therapy. 

In order to get any adequate conception of the possibilities 
of therapeutic results in cases of neurosyphilis, one must 
consider the pathological changes that occur and how far 
these changes are reparable. In cases in which the destruc- 
tion of tissue is marked, it is, of course, out of the question to 
expect to get any marked clinical improvement. A case of 
spastic hemiplegia * in paretic neurosyphilis is given with the 
autopsy findings as an illustration of irreparable damage 
that may occur to the parenchymatous structure, thus pre- 
cluding any chance of functional recovery. 

On the other hand, there is a group of cases in which the 
symptoms may be exceedingly severe and yet the actual de- 
struction of tissue be almost nil. This point is illustrated by 
a case f in which total duration of symptoms terminating in 
death was only 22 days. At autopsy there was very little in 
the way of macroscopical lesions, and microscopically there 
was no marked evidence of destruction in the parenchymatous 

* James McDevitt (99). 
t Jacob Methuen (100). 



SUMMARY AND KEY 459 

tissue. The lesions were represented chiefly by perivascular 
infiltration. According to all our modern ideas, this type of 
reaction is resolvable under antisyphilitic treatment. Though 
this case was one of very short duration, similar pathological 
pictures may be obtained in cases of considerably longer 
standing. It is also of great importance to remember that 
symptomatically such a case may be in no way distinguished 
from a case with marked atrophy. 

Another autopsied case is given which shows an exceedingly 
marked meningitis.* The meningitic processes according to 
the literature and experience react very readily to antisyphi- 
litic treatment in the form either of mercury and iodid or in 
combination with salvarsan. The lesion here present would 
probably have improved had intensive treatment been given. 
Clinically the diagnosis of general paresis was made and, as 
has been the rule in the past, treatment was not given on 
the ground that it had no value in paresis. While this is an 
extreme case of meningitis, it is to be remembered that the 
vast majority of cases of paretic neurosyphilis show some de- 
gree of meningitis. Just as in the marked meningitis of the 
diffuse neurosyphilis, so with the meningitis of the paretic 
form, improvement is expected under treatment. As a part 
or even the whole of the symptomatology in a given case may 
be due to this meningitic process, we have reason occasionally 
to expect marked improvement as the result of antisyphilitic 
treatment. 

As a contrast to this case with marked meningitis, another 
case of marked atrophy f is given. Here the atrophy was very 
perceptible. on macroscopical examination and the mere view 
of the brain at once indicated that in such a case important 
results from treatment were not to be expected. 

The topographical variation of the lesions in neurosyphi- 
lis must be remembered when treatment is to be instituted. 
Thus very marked lesions may exist in portions of the brain 
which do not give any very definite localizing symptoms. As 
a result, one may be led to believe from clinical evidence 
that the case is a very mild one though the lesions may 

* John Baxter (loi). f Theodosia Jewett (102). 



460 SUMMARY AND KEY 

really be very extensive. The topographical distribution 
must, therefore, be taken into consideration in trying to 
estimate the damage done. This point of topographical dis- 
tribution of the lesions is illustrated by a case.* 

It has been generally recognized that clinical improvement, 
if not cure, may be readily obtained in the group of diffuse 
neurosyphilis, i.e., so-called cerebral and cerebrospinal forms 
of syphilis. These are cases in which the parenchyma is very 
slightly, if at all, affected and in which the lesion is chiefly 
in the meninges and blood vessels, irritative rather than 
degenerative. A case f is given to illustrate this point. In 
our experience systematic intravenous salvarsan therapy 
associated with mercury and iodid gives remarkably good 
results in the vast majority of this group of cases. 

It is generally conceded that antisyphilitic treatment, 
particularly salvarsan, has a very satisfactory result applied 
to diffuse neurosyphilis. But the same good results may be 
obtained in cases which are not so typically of the diffuse 
type. An illustration is given in the case of a machinist in 
which the diagnosis was in doubt between paretic, tabetic or 
diffuse neurosyphilis. J The result of treatment was as satis- 
factory as could be expected in any type of neurosyphilis 
and this in a case of several years' duration with Argyll- 
Robertson pupils. 

As a rule, the Argyll-Robertson pupil is taken as a grave 
omen for treatment, an idea based upon a conception that 
the Argyll- Robertson pupil so frequently represents the old 
so-called " parasyphilitic " cases, which, in the past were 
taught as being incapable of improvement by the ordinary 
antisyphilitic methods. 

A second case § with Argyll-Robertson pupil shows again 
that the prognosis may be very good despite the Argyll- 
Robertson sign. 

* A. W. (103). Agnes O'Neil (19), 

t John Edwards (104). Cf. ^^^^^ ^^"^^ ^^^'>' 

Henri Lep^re (105), X Henri Lepere (105). Cf. 
Frederick Stone (106), Julius Kantor (54). 

Arthur Bright (121), g Frederick Stone (106). 



SUMMARY AND KEY 46I 

But even in the diffuse neurosyphilis, the symptomatic re- 
sults of treatment may not be entirely happy. Under treat- 
ment it may be possible to reduce the spinal fluid tests to 
negative without, however, as in the case of our hemiplegic 
lady,* making the physical or mental symptoms disappear. 
In other words, it may be possible to stop the active prog- 
ress of the disease without removing the symptoms. 

One is always warned of the danger of intravenous salvarsan 
therapy in hemiplegic cases due to arteriosclerotic conditions. 
While this warning is well justified, it does not mean that the 
most intensive treatment is contraindicated, as shown in the 
case of our hemiplegic machinist. f Such may be given over 
long periods of time with the most satisfactory results. 

A case | is given which illustrates the value of antisyphilitic 
treatment in cases showing symptoms of intracranial pres- 
sure due to syphilitic disease. In the case of the woman which 
we cite, we believe that the symptoms of intracranial pressure 
were probably due to a gummatous new growth, although it is 
possible that they were due to a marked meningitic process. 
However, the results of a limited amount of antisyphilitic 
treatment in this case were very brilliant. Similar results 
may often be obtained in gumma of the brain. This is not 
always true, however, and it may become necessary to use 
surgical procedure in order rapidly to overcome the effects of 
intracranial pressure. 

While it has always been conceded that treatment would 
greatly help cases of diffuse and vascular neurosyphilis, the 
utmost pessimism has existed concerning the results to be 
obtained by treatment in cases of tabetic and paretic neuro- 
syphilis. Only in the last five or six years, due to the stimulus 
of Ehrlich's discovery of salvarsan and the introduction of the 
intraspinous methods of therapy, have intensive work and 
study been given to the treatment of these cases. And though 
it has been by no means settled in the minds of the various 
workers in this field, as to what the ultimate results of such 



*^ Greta Meyer (107). CJ. f Victor Friedburg (108). 
John Jackson (14). % Annie Rivers (109). 



462 SUMMARY AND KEY 

treatment will be and though some do not believe that there is 
any good to be expected from our present methods, still the 
majority of men who are treating these cases systematically 
feel very much encouraged. 

At times very brilliant results are to be obtained by in- 
traspinous treatment in tabetic neurosyphilis (" tabes dor- 
salis "). A very striking illustration is given of a case of 
this sort in which the symptoms dated only a few months but 
which had all the classical symptoms, signs and laboratory 
tests. Five intraspinous injections of mercurialized serum 
were sufficient to cause the disappearance of the subjective 
symptoms and to reduce the spinal fluid test to negative.* 

It must be emphasized that the best results in cases of 
tabetic neurosyphilis are usually to be expected in cases in 
which the symptoms are of short standing. Where the proc- 
ess is of long duration and much destruction of spinal cord 
tissue has occurred, the best one can expect is that the ac- 
tivity and progress may be halted. This is illustrated by our 
case of a baker, 43 years of age, who had been suffering from 
the symptoms of tabes for some years. Under treatment it 
was possible to get an entirely negative serology of the blood 
and spinal fluid. f Despite this evidence that the activity 
of syphilis had ceased, the symptoms continued unabated. 
We are ready to believe, however, that much good was ac- 
complished. For the patient should not have any further 
untoward developments or the appearance of any new 
symptoms. These, without such treatment, might well be 
expected. At times excellent clinical results are obtained in 
long standing cases. 

The results of treatment in paretic neurosyphilis (" general 
paresis ") have been considered even less hopeful than in 
tabetic neurosyphilis ("tabes dorsalis ") ; indeed, it has 
often been stated that the patients are made worse by 
treatment. Recent work, however, supports a much more 
optimistic viewpoint. We feel that intensive treatment has 
been of the greatest value in a number of cases of paretic 

* Mr. McKenzie (no). Cf. f Ivan Rokicki (in). 
Ivan Rokicki (in). 



SUMMARY AND KEY 463 

neurosyphilis. Two cases are given which show the most satis- 
factory and brilhant results of intensive intravenous salvarsan 
therapy in cases diagnosed as general paresis. The first case, 
an excellent salesman, 46 years of age, with most aggravated 
mental symptoms, recovered symptomatically and all his 
tests were rendered negative.* He has now remained entirely 
well and economically efficient for about two years without 
further treatment. The other case, f a housewife, also with 
very marked symptoms suggestive in all ways of general 
paresis, also recovered rapidly under treatment and her 
tests became negative. Her remission has now lasted for 
nearly three years without further treatment. 

At times it is not possible to get the spinal fluid tests to 
become negative in cases of paretic neurosyphilis under the 
most intensive salvarsan therapy. In spite of this, the 
clinical condition of the patient may improve so greatly that 
the patient can be considered clinically recovered. An illus- 
tration is given of an undertaker J who was brought from a 
condition of the greatest cachexia and mental confusion to a 
condition of robust appearance and mental efficiency under 
intravenous salvarsan therapy, in spite of the fact that his 
tests were very slightly if at all reduced in intensity. He 
has been able to resume his former occupation and his former 
life with great satisfaction to himself and his family. 

Improvement in paretic neurosyphilis under treatment is 
not to be expected very early. Two or three months of 
active treatment may elapse before one sees signs of improve- 
ment. Indeed, as illustrated by our case of the shipping clerk, 
this improvement may begin to make its appearance only 
after more than four months of intensive treatment consisting 
of two injections of salvarsan per week. § In spite of the long 
delay in this case, complete clinical recovery occurred and the 
tests became almost negative at the end of a year of treatment. 

* Albert Forest (112). Cf. Levi Morovitz (122), 
Gussie Silverman (113), Peter Burkhardt (58). 

^^m!-^^' ^^""'^^ V'^^i^ t Gussie Silverman (113). 

William Rosetti (116), + ,,, ,^ „ . x 

Annie Martin (117), t Walter Henry (114). 

§ Henry Ryan (115). 



464 SUMMARY AND KEY 

It is not only in the central nervous system that the 
syphilitic process may resist the most intensive treatment. 
In the case of the speculator, a victim of paretic neurosyphilis, 
which we cite, a perennially recurrent iritis appeared after 
several months of the most intensive salvarsan treatment 
which was apparently sufhcient to reduce the symptoms of 
the paretic neurosyphilis,* but not of non-neural syphilis. 

We give the case of a charwoman having the diagnosis of 
paretic neurosyphilis, who, under intensive treatment, made a 
symptomatic recovery. The interesting point in her findings 
is that all the tests in the spinal fluid became negative except 
the gold sol reaction which remained of the " paretic " type.f 
There is no general rule as to the reaction of the spinal fluid 
tests under treatment. At times one test is the first to disap- 
pear under treatment; again it is another. We have seen 
many cases in which the gold sol was the first test to become 
negative and others, as the case given, in which it is the last to 
show any change. As in our undertaker, symptomatic clini- 
cal improvement may be practically complete without any 
change in the spinal fluid tests. 

One must remember that it is the condition of the patient 
that is of first importance; not so much the laboratory tests. 
Having shown the clinical recoveries with the tests remaining 
positive, we now have to report two cases in which there 
was improvement as shown by the tests but no clinical im- 
provement. The first patient, a bank teller| of 39 years, with 
a diagnosis of paretic neurosyphilis, received Intensive Intra- 
venous salvarsan for several months. Under this treatment 
all the tests became negative except the gold sol which re- 
mained of the paretic type. In spite of this, there was not 
the slightest improvement In his mental condition. 

The second case, a young man of 29 years in whom the symp- 
toms of neurosyphilis had recently appeared, under treatment 
showed a marked diminution in the intensity of the spinal 



* William Rosetti (116). J William Roberts (118). 

. ,-r . / ^ ^. John Silver (119). 

t Annie Martin (117). Cf. *' ^ ^ 
William Roberts (118). 



SUMMARY AND KEY 465 

fluid tests, notwithstanding which the patient became more 
and more demented and died after a series of convulsions.* 

Of course, good results indicated above in some of our cases 
of paretic neurosyphilis are not to be expected in every case 
no matter how intensive the treatment. We give a case of 
paretic neurosyphilis in which the most intensive intravenous 
salvarsan therapy gave no satisfactory results. This was 
followed by several intraventricular injections of salvarsan- 
ized serum. The results of this combined treatment, however, 
were still not satisfactory, and the patient died.f 
[fi^In order to emphasize as strongly as possible what we be- 
lieve is a great advantage of systematic intensive treatment 
for neurosyphilis, we offer two cases in different time periods 
of neurosyphilis. The first is a printer with the symptoms of 
diffuse neurosyphilis six months after the appearance of his 
chancre. J These symptoms appeared despite three injections 
of salvarsan, injections of mercury and mercury by mouth. 
Under intensive treatment (meaning injections of salvarsan 
twice a week and continued injections of mercury), complete 
recovery occurred in a few weeks. 

The second case is that of a waiter with signs and symptoms 
of neurosyphilis in whom the diagnosis lay between the diffuse 
and paretic forms. § This patient developed his symptoms 
in spite of continuous antisyphilitic treatment during the six 
years since his infection. This treatment had been compar- 
atively mild, consisting in great part of mercury by mouth. 
However, he had had courses of injections of mercury and 
several injections of salvarsan. Under a systematic course of 
intravenous injections of salvarsan twice a week for a number 
of months, all symptoms disappeared and the spinal fluid tests 
became negative as well as the W. R. in the blood serum. 

A final case is offered which indicates that antisyphilitic 
treatment may occasionally be of service in improving the 
mentality of a Feebleminded Congenital Syphilitic] | 

* John Silver (1x9). John Bennett (34). 

f James McGinnis (120). § Levi Morovitz (122). 

t Arthur Bright (121). Cf. 1 1 Robert Matthews (23) . Cf. 
Levi Morovitz (122), Isaac Goldstein (37). 



466 SUMMARY AND KEY 

No attempt has been made in this section to give a per 
cent evaluation of the results of treatment in any one group of 
neurosyphilis. Two charts (charts 25 and 26), however, are 
appended which give an indication of some of our results. It 
seems to us, however, that it is too early to make any definite 
statements as to how far treatment will take us in the groups 
of neurosyphilis. We do feel decidedly, however, that many 
patients, in whatever group of neurosyphilis the diagnosis 
may place them, will respond to intensive systematic anti- 
syphilitic treatment. It is unfair to give an entirely grave 
prognosis in any case of neurosyphilis until the effect of 
treatment has been tried. 

In a separate section, entitled NEUROSYPHILIS AND 
THE WAR, we have presented fourteen cases selected from 
British, French and German writers in the war literature of 
1914-16. Most of these cases were naturally somewhat inad- 
equately reported under the critical conditions of literature 
made in the war. We present the cases for what they are 
worth : at all events they draw attention to the extraordinary 
interest of the neurosyphilis problem in relation to the war. 

Such cases as A, one of tabes dorsalis apparently develop- 
ing paresis by a process akin to shell-shock, is of value in the 
interpretation of the development of paresis in civil life. 
By " shell-shock " we commonly refer to a condition in which 
there is no actual traumatic injury of the brain. The hy- 
pothesis must be then that the explosion in some way indi- 
rectly caused an alteration of living conditions of the spiro- 
chetes, permitting the development of paresis. 

Case B similarly seems to be a case in which a latent syphi- 
lis has turned shell-shock into tabes dorsalis. 

Cases C, D, E bring up the question of aggravation of 
neurosyphilis by service and on service, respectively. 

Case F likewise shows how, in the determination of amount 
of pension, the probable duration of the neurosyphilitic pro- 
cess is important. 

Case G seems to shoW that war stress"! alone, without the 
emotional or physical effects of shell-shock, may kindle a 
latent syphilis into paretic neurosyphilis. 



SUMMARY AND KEY 467 

Case H similarly suggests that- (the "gassing" process may 
effect the same result. 

Case I seems to show that the neuropathically tainted 
person may have latent epilepsy brought out through syphilis, 
the syphilis in this case having been acquired during the first 
summer of the war. 

Case J was an interesting case of a syphilitic who, after 
the stress of the Battle of Dixmude, became an epileptic. 

Syphilitic root-sciatica was developed in Case K at work 
in the war zone. 

Case L is one of a civilian who apparently would not have 
developed paresis at precisely the moment when he did, if 
he had not been discharged as a German Jew from his long- 
held bank position in London. 

Two cases, M and N, are cases of shell-shock, non-syphi- 
litic; yet the picture of paresis in the one case and of tabes 
in the other was for a long time almost convincing to the 
examiners. They are better termed cases of pseudoparesis 
and pseudotabes, using the prefix "pseudo", as usual, to 
signify a non-syphilitic imitation of the disease in question. 

To sum up in the most general way the lessons of this 
book, we may emphasize again (i) the unity-in-variety of the 
phenomena of neurosyphilis, (2) the value of a hopeful approach 
to the therapy of all cases of neurosyphilis, even the paretic form, 
and (3) the value of applying syphilis tests to every case of neuro- 
sis or psychosis. 

(i) Re unity-in-variety of neurosyphilitic phenomena. 

The unity of these phenomena is confirmed, theoretically, 
by the common factor of spirochetosis: practically, by the 
Wassermann reaction, positive in serum or spinal fluid! Al- 
most at this point the unity of phenomena ceases. Neither 
chronicity, nor evidence of mononuclear cell-deposits, nor 
evidence of serious structural damage to the nervous system, 
nor presence of other positive tests than the W. R.,* nor 

* For cases in which, without autopsy we have risked the 
diagnosis neurosyphilis in the absence of W. R. in serum or 
fluid, see William Twist (13), Frederick Wescott (18), Martha 
Bartlett (21), Thomas Donovan (23), Paolo Marini (28), 
Margaret Neal (32), Bridget Curley (59), Victor Friedburg 
(108), Ivan Rokicki (iii). 



468 SUMMARY AND KEY 

existence of mental or nervous symptoms or signs, is a com- 
mon feature of neurosyphilis. Sometimes the nervous system 
appears to harbor spirochetes in the most cordial manner as 
guest-friends {paresis sine paresi.) Again, perhaps as an 
expression of elaborate processes of immunity, the spi- 
rochetes take effect in relatively huge gummata. Sometimes 
the neurosyphilitic process rises as if by a regular process of 
siege from spinal nerve-root to spinal nerve-root (tabes 
dorsalis and diffuse neurosyphilis). Again, the nervous 
system is taken by storm, as it were (disseminated encepha- 
litis). Very frequently the neurosyphilis is simply an in- 
direct effect of blood-vessel disease, and huge masses of 
tissue are scooped out in necrosis with dependent secondary 
degenerations ; and later the extinct lesions of vascular origin 
may or may not betray evidence of their syphilitic origin. 
Sometimes diffuse processes run on, apparently, with perfect 
fatalism to a mortal issue in a few years both with and without 
treatment. Again treatment appears to accomplish much 
(see fuller discussion under 2). The laws governing the pref- 
erence of processes to lodge in membranes, vessels, and 
parenchyma, and in all combinations of these, have not been 
worked out. Hardly a case of neurosyphilis, properly studied 
ante mortem and post mortem, but would throw important 
light on our medical approach to one of the great problems 
of civilization, the problem of syphilis as a whole. 

(2) Re value of a hopeful approach to the therapy of neuro- 
syphilis. 

The prognosis of neurosyphilis is not worse than that of 
the chronic diseases in general. In fact, the prognosis of 
neurosyphilis quoad vitam is either good or dubious, certainly 
not bad. The surprising reversals of form which the spi- 
rochete shows in certain remissions are always to be awaited. 
Treatment of neurosyphilis has certainly effected amazing 
results, not so much by way of Ehrlich's therapia sterilisans 
magna as by means of systematic intensive treatment. 
Even paretic neurosyphilis (general paresis) seems to have 
been cured. Preparetic phases are theoretically hopeful. 
Nor is it so certain that paretic neurosyphilis will ultimately 
prove a perfectly distinct species of neurosyphilis. General 



SUMMARY AND KEY 4^9 

paresis seems to us at least to be more closely related to diffuse 
neurosyphilis than is tabes dorsalis to diffuse neurosyphilis. 
In any particular case, moreover, during a good part of the 
early months or years, it is difficult or impossible to tell the 
paretic from the non-paretic forms of diffuse neuros3rphilis 
by any combination of clinical observations and tests. In 
the instance of more protracted neurosyphilis, e.g., tabetic, 
the outlook for vascular complications is such that antisyphi- 
litic treatment directed at prevention of these complications 
is scientifically warrantable, even if the tabetic process itself 
proves unassailable. The old distinction of syphilis and 
parasyphills, so striking and apparently satisfactory when 
introduced by Fournier, seems to be a false distinction which 
should be dropped. Therapeutically, we should approach 
all cases of neurosyphilis without bias or nihilistic prejudg- 
ments. 

(3) Re universal applicability of syphilis tests in nervous 
and mental cases. 

The importance of putting every neurosis or psychosis 
through syphilis tests is not based alone on the frequency of 
neurosyphilis, though neurosyphilis is surely frequent enough. 
The importance of universally applying these tests is estab- 
lished by the experience of lingering doubts both in the phy- 
sician's mind and (nowadays increasingly) in the patient's 
and friends' minds, so long as these tests are not applied. 
Nor should the positive serum Wassermann reaction fall to 
be followed by lumbar puncture and appropriate tests. The 
general practitioner confronting neuroses or psychoses — and 
what practitioner does not? — must not expect valuable 
results from consultation with neurologists and psychiatrists 
when he does not carry to these specialists the results of at 
least the serum W. R. In his patient. Not only are prac- 
titioners, specialists, and patients subject to discomfiture on 
the eventual and delayed proof of syphilis or neurosyphilis, 
but valuable time has been lost to treatment. How often 
the physician of yore (and really not so long since) had to be 
regarded as an eccentric virtuoso If he tested urine as routine! 
Well, for routine use in nervous and mental diseases, the 
Wassermann serum reaction is at least as important as urin- 



470 SUMMARY AND KEY 

analysis. Nor would we cease our homily with the general 
practitioner. We know neurologists and psychiatrists who 
use the Wassermann test only when it is likely to be positive! 
But they are dying out. 



APPENDIX A 

In appendix A a brief outline is given of the six tests (W. R. 
on blood serum and spinal fluid, cell count, globulin test, 
albumin test, gold sol test). This is not intended as a com- 
plete working manual but rather as indicating the methods 
used in diagnosis in the cases presented herein. For more 
complete details the reader may be referred to textbooks 
on the subject of serology, among which may be mentioned 
Kaplan: "Serology of the Nervous System"; Plaut, Rehm 
and Schottmiiller: " Leitfaden zur Untersuchungen der 
Zerebrospinalfiiissigkeit " ; Kolmer: "Infection, Immunity 
and Specific Therapy," and, for the Wassermann technique, 
an article by Dr. W. A. Hinton in M, J. Rosenau's " Pre- 
ventive Medicine and Hygiene." 

Our own W. R's. have been performed at the Wassermann 
laboratory of the Massachusetts State Board of Health 
(formerly the Neuropathological Testing Laboratory, Har- 
vard Medical School), under the supervision of Dr. W. A. 
Hinton. The other tests are performed at the Psychopathic 
Hospital. It is very important that a close relationship should 
exist between the clinician and the Wassermann laboratory 
if the most is to be obtained from the reactions. This rela- 
tionship has been effectively close between the authors and 
the above-mentioned laboratory; and has enabled us to get 
very much clearer ideas about certain cases than could other- 
wise have been obtained. 

Cell Count. In order to obtain the number of cells per 
cmm., the examination should be made of the fresh fluid as 
soon as possible after this is withdrawn. The most con- 
venient counting chamber for this purpose is the so-called 
Fuchs- Rosenthal counting chamber, the ruled spaces of which 
contain slightly over 3 cmm, (an ordinary blood cell counting 
chamber may be used). According to the method used by 
us the cells are stained in a pipette with Unna's polychrome 
methylene blue. Using a white-counting pipette, stain is 

471 



472 APPENDIX A 

drawn up to the first or second marking and the remainder of 
the pipette filled with spinal fluid. This makes no change 
in the dilution for practical purposes. After two or three 
minutes the staining is satisfactory and the counting may 
be done. With this stain a differential count may be made. 
Plasma cells stain a lavender as contrasted to the blue of 
the lymphocytes. The characteristic halo surrounding the 
eccentric nucleus is visible. The blood cells do not assume 
color with this stain; hence it is unnecessary to add any acetic 
acid. 

For permanent preparations, and more accurate differ- 
ential counts of the spinal fluid, the Alzheimer method may 
be used. The technique is given in a paper by H. A. Cotton 
and J. B. Ayer as follows: * 

1. Lumbar puncture in the usual manner. 

2. 96% alcohol, in proportion to twice the amount of 
cerebrospinal fluid, is added drop by drop and well mixed. 

3. Centrifuge the mixture for one hour at high speed in a 
glass tube with conical end. (An ordinary electric urinary 
centrifuge apparatus can be employed, the tube to be well 
stoppered to prevent evaporation.) 

4. The supernatant fluid is poured off, leaving a small 
coagulum in the bottom of the tube. 

5. Add absolute alcohol — alcohol and ether — ether, each 
separately for one hour, to dehydrate and harden coagulum. 

6. The coagulum can now be gently loosened from the 
bottom of the tube by a long needle. The tube is then in- 
verted, and the coagulum allowed to fall into the hand by a 
quick tap on the end of the tube. Care must be taken not to 
squeeze or handle the coagulum. The hand is placed over 
a small homeopathic vial, containing thin celloidin, and the 
coagulum allowed to drop into the celloidin, where it remains 
over night (twelve hours usually). 

7. Coagulum is placed in thick celloidin which is allowed 
to evaporate slowly. It is then mounted on blocks and 
sections cut 14^ in thickness. 

* From Mallory and Wright: Manual of Laboratory Tech- 
nique. 



APPENDIX A 473 

8. The sections are stained and mounted according to 
the following procedure : 

(a) Remove celloidin by absolute alcohol and ether. 

(6) 8o% alcohol. 

(c) Water. 

{d) Sections are carried on glass or platinum needle into 
a dish of Pappenheim's pyronin-methyl green stain and kept 
in a water-bath at 40° C. five to seven minutes. 

(e) Quickly cool dish in running water. 

(/) Wash off superfluous stain in plain water. 

(g) Absolute alcohol to differentiate — until no more 
stain comes away from section. 

(h) Clear in Bergamot oil. 

(i) Mount in balsam. 

The normal cell count may be stated as being up to 6 cells 
per cmm. ; from 6 to 12 cells may be considered as suggestive 
of pathological condition and more than 12 cells per cmm. as 
definitely pathological. The type of cell in syphilitic diseases 
is preponderantly the small lymphocyte. A low percentage, 
that is, very rarely over 20%, of large lymphocytes, endo- 
thelial phagocytic cells, polymorphonuclear leucocytes and 
plasma cells may also be found. The finding of plasma 
cells in any number in the spinal fluid is suggestive although 
not conclusive evidence for the diagnosis of paretic neurosy- 
philis. 

Globulin is an albumin which is precipitated by half satura- 
tion with a salt. A very simple and satisfactory test is known 
as the Nonne-Appelt test, which has been modified by Ross- 
Jones. Into a test-tube of small diameter, run i cc. of spinal 
fluid. Place under this fluid with a pipette, i cc. of a satu- 
rated solution of ammonium sulphate ((NH4)2S04). If any 
globulin is present a white, sharply-defined ring will form at 
the junction of the two fluids. According to our readings, a 
ring that is just visible with the aid of a black background is 
called i + , a ring that is just visible without the black back- 
ground, 2+; a ring easily perceptible, 3-I- and a relatively 
very heavy ring, 4-I-. On shaking the tube, if globulin is 
present, the fluid will show turbescence. 



474 APPENDIX A 

Another simple globulin test used In our laboratory as a 
check on the Nonne-Appelt test Is the Pandy test. A few 
cc. of a clarified io% solution of phenol are placed In a watch 
glass. One drop of spinal fluid Is run Into this solution. A 
milky turbescence Indicates globulin. 

The presence of globulin in the spinal fluid Is always an 
indication of abnormality of the cerebrospinal axis. There 
is nothing differential In this finding as It occurs in all inflam- 
matory processes. However, it is characteristically present 
In most cases of neurosyphilis (exception to the rule: the pure 
vascular type does not show globulin In a very high per cent) . 

Albumin Test. Albumin in small quantities is present 
in all spinal fluids. Increase over the normal amount occurs 
in pathological conditions such as most cases of neurosyphi- 
lis, especially In those In which globulin is found. Any al- 
bumin precipitant may be used for rough clinical calculation, 
comparing the amount of precipitate with that from the nor- 
mal fluid. Our method is to place i cc. of spinal fluid In a 
small test tube of about 5 mm. diameter and to precipitate the 
albumin by the addition of 3 drops of 33i% of trichloracetic 
acid. This test has Its chief value as confirmatory of the 
globulin test, since in the vast majority of instances where 
globulin Is found there will also be found an increase In 
albumin. 

The Gold Sol Reaction is an empirical test discovered by 
Carl Lange in the utilization of the work of Zslgmondl with 
solutions of colloidal gold and albumins. Briefly the details 
of the test are as follows: 

Ten tubes are set up In a rack. To the first tube 1.8 cc. of a 
0.4% of salt solution Is added and to each of the following 
tubes I cc. of this solution. Then to the first tube containing 
1.8 cc. of salt solution one adds 0.2 cc. of the spinal fluid to be 
tested. This gives a dilution of i to 10. From this tube i 
cc. is pipetted into the second tube and this process continued 
through the ten tubes. This gives dilutions of spinal fluid 
of I to 10, I to 20, I to 40, etc., to I to 5120 In the last tube. 
Then 5 cc. of colloidal gold solution is added to each tube. 
A positive reaction Is Indicated by the precipitation or 
throwing down of the colloidal gold into its metallic form. 



APPENDIX A 475 

This produces a change In color. This precipitation may 
be partial or complete and the amount of precipitation is 
indicated by the color and is read as follows : 

The unchanged fluid is called o; a slight change giving a 
red-blue as i; a further change giving a blue-red as 2; a 
straight blue as 3 ; a lavender or violet as 4 ; and the colorless 
fluid representing complete precipitation as 5. The num- 
bers are placed in a row, indicating the tube in which the 
color occurs. The fluid from a case of paretic neurosyphilis 
will give a complete precipitation beginning in the first tube 
and running through a number of tubes and then grading off. 
It may be indicated 5555431000. The characteristic 
reaction of fluids from tabetic and diffuse neurosyphilis is 
less strong than from the paretic. The greater part of the 
reaction will take place, however, in the first five tubes, but 
as a rule it will not begin very strongly in the first two. 
A characteristic reaction is 1233210000. Another 
reaction that may be considered characteristic of the tabetic 
or diffuse form is 3332100000. Fluids from non- 
syphilitic cases as a rule give a reaction having its greatest 
intensity beyond the fifth tube, that is, in the high dilutions. 

A reaction characteristic of brain tumor or tuberculous 
meningitis is 0000133210. 

The conclusions that may be drawn from the gold sol 
reaction have been summarized by one of the authors as 
follows : 

1. Fluids from cases of general paresis will give a strong 
and fairly characteristic reaction, especially if more than one 
sample Is tested, in the vast majority of cases. 

2. Very rarely a general paresis fluid will give a reaction 
weaker than the characteristic one. 

3. Fluids from cases of syphilitic Involvement of the 
central nervous system other than general paresis often give 
a weaker reaction than the paretic, but In a fairly high per- 
centage of cases give the same reaction as the paretics. 

4. Non-syphilitic cases may give the same reaction as the 
paretics; these cases are usually chronic Inflammatory con- 
ditions of the central nervous system. 

5. When a syphilitic fluid does not give the strong " pa- 



476 APPENDIX A 

retic reaction," it is good presumptive evidence that the case 
is not general paresis; and this test offers a very valuable 
differential diagnostic aid between general paresis, tabes and 
cerebrospinal syphilis. 

6. The term " syphilitic zone " is a misnomer, as non- 
syphilitic as well as syphilitic cases give reactions in this 
zone; but no fluid of a case with syphilitic central nervous 
system disease has given a reaction out of this zone (test 
thus valuable negatively). Any fluid giving a reaction out- 
side of this zone may be considered non-syphilitic. 

7. Light reactions may occur without any evident sig- 
nificance, while a reaction of no greater strength may mean 
marked inflammatory reaction. 

8. Tuberculous meningitis, brain tumor and purulent 
meningitis fluids characteristically, though not invariably, 
give reactions in higher dilutions than syphilitic fluids. 

9. The unsupplemented gold sol test is insufficient evi- 
dence on which to make any diagnosis, but used in conjunc- 
tion with the Wassermann reaction, chemical and cytological 
examinations, it offers much information looking toward 
the differential diagnosis of general paresis, cerebrospinal 
syphilis, tabes dorsalis, brain tumor, tuberculous meningitis, 
purulent meningitis. 

10. We believe that no cerebrospinal fluid examination 
is complete for clinical purposes without the gold sol test. 

The Wassermann reaction as carried out in the|Wasser- 
mann Laboratory is based on the principles of the original 
method — the only essential modification consists in the em- 
ployment of cholesterinized alcoholic extracts of human 
hearts as antigen instead of aqueous extracts of foetal livers 
from cases of congenital syphilis. Experience has shown that 
properly standardized antigens made from human hearts are 
much more sensitive in the detection of true cases of syphilis. 

Antigens. Three antigens are used, each being an alcoholic 
extract of human heart which is saturated at room tempera- 
ture with cholesterin. These antigens differ slightly in their 
sensitiveness. Before the test is made each antigen is diluted 
with 0.85% salt solution in the proportion of four parts of 



APPENDIX A 477 

the cholesterinized antigen extract to sixteen parts of 0.85% 
salt solution. The amount to be used, the dosage, is care- 
fully determined by testing each antigen against a large 
number of known positive and known negative specimens of 
blood. The dosage of the antigens employed is less than one- 
half the amount which inhibits hemolysis when the antigen is 
incubated for one hour with the hemolytic system which 
consists of complement, amboceptor and cells in the proper 
proportions. These antigens are designated as A, B, and C. 
Antigen A is the most sensitive. B and C are very similar 
to each other quantitatively and qualitatively. 

Specimens to be tested. The serum which separates from 
the clot is withdrawn, centrifugalized if necessary, and then 
heated at 55 degrees for thirty minutes, o.i cc. of serum 
is used in the test and 0.2 cc. of each specimen is used as 
a control to exclude the presence of anti-complementary sub- 
stances. Spinal fluids are tested in two ways. As a routine 
0.5 cc. of the spinal fluid is used in the test and i.o cc, is 
used in the control ; or when especially requested spinal fluids 
are titrated by using respectively i.o, 0.7, 0.5, 0.3, and o.i cc. 
of the spinal fluid for each test and 1.0 cc. of spinal fluid 
for the control. Spinal fluids are not inactivated. 

Complement. The complement is obtained from the serum 
of guinea pig's blood. No complement is used when older 
than eighteen hours. A 10% solution and 0.85% salt solu- 
tion is used in the test. The amount used is twice the mini- 
mum quantity necessary to hemolyze the sensitized cells. 

Sheep's Corpuscles. A 5% suspension of sheep's corpus- 
cles in 0.85% salt solution is prepared from defibrinated 
sheep's blood. The corpuscles are washed three times and 
for each washing four to five times as much 0.85% salt 
solution is used as the original volume of the defibrinated 
blood. 

Amboceptor. The amboceptor is prepared by injecting 
sheep's corpuscles into a rabbit. The serum of this rabbit 
which contains amboceptor is diluted with 0.85% salt solution 
so that 0.25 cc. will hemolyze 0.5 cc. of a 5% suspension of 
sheep's corpuscles. In the test twice the quantity or 0.5 cc. 
of amboceptor is used. 



478 APPENDIX A 

Sensitized Cells. The sensitized cells consist of equal 
parts of washed sheep's corpuscles and diluted amboceptor. 
This mixture is incubated in a water bath at 37° C. for a 
half hour to effect the sensitization of the cells. 

Technique of the Wassermann Test. One-tenth cubic 
centimeter of each inactivated specimen of serum and 0.5 cc. 
of each uninactivated specimen of spinal fluid is pipetted into 
a separate tube. A mixture is freshly prepared in salt solu- 
tion, each cubic centimeter of which contains the proper 
amount of antigen A (the most sensitive antigen), and two 
units of a 10% solution of guinea pig serum (complement). 
One cubic centimeter of this mixture is pipetted into each 
test tube. These tubes are then incubated for forty minutes 
in a water bath at 37° C. At the end of this period, sensi- 
tized cells are added, and the tubes are again incubated in a 
water bath at 37° C. for one hour. Each specimen which 
shows any degree of inhibition of hemolysis is retested in 
the afternoon. For this second test antigen A is again used 
and in addition antigens B and C. A control is also made 
for each specimen retested to eliminate any possibility of 
the inhibition of hemolysis being due to anti-complementary 
substances in the serum or spinal fluid tested. The technique 
of the second test differs in no wise from that of the first, 
except for the use of a control in each retested specimen 
and the employment of three antigens instead of one. The 
degree of positiveness is noted for each retested specimen and 
compared with the degree of positiveness obtained for the cor- 
responding specimen with the same antigen-complement-salt 
solution mixture in the morning's test. The specimen is re- 
tested on the next day when discrepancies occur between the 
morning reading for antigen A and the afternoon reading 
for antigen A. From the above description it will be noted 
that the negative specimens have but a single test with one 
antigen only, while the positive specimens are retested, thus 
permitting a confirmation of any positive reaction. In this 
way attention is focalized on the positive specimens. 

Interpretation of Results. Antigen C (the weakest of the 
three antigens) is used entirely for diagnostic purposes and 
any specimen showing the slightest degree of inhibition with 



APPENDIX A 479 

this antigen and stronger degrees of inhibition with the other 
antigens is reported as positive. The specimens which are 
strongly or moderately positive with antigens A and B and 
negative with antigen C are reported as doubtful. In testing 
spinal fluids by the titration method, antigen C is used and 
the readings are based upon the degree of inhibition of hemol- 
ysis noted. The intensity of this inhibition is indicated by 
Arabic numerals: " 5 " indicates complete inhibition, 
while " I " means a faint cloudiness, hence a weak reaction. 
Intermediate numbers show relative intensity varying be- 
tween complete inhibition " 5 " (strong positive) and slight 
inhibition "i " (weak positive); " — " equals no inhibition 
(negative). 

Although it is commonly believed that the recent ad- 
ministration of antisyphilitic treatment will affect the reaction 
by making it negative, this is not our experience, and it is, 
therefore, not necessary that treatment be withdrawn for a 
short period before the specimen is submitted for examination. 

The reaction as carried out in this laboratory has the 
following diagnostic significance: Positive indicates sjrphilis, 
except very rarely in acute febrile conditions such as malaria 
and pneumonia. Negative does not exclude syphilis. In 
obscure conditions a series of less than three negatives has 
little diagnostic significance. Doubtful suggests syphilis. 
It is therefore advisable to submit three or more specimens 
in such a case, and interpret a persistently or predominat- 
ingly doubtful reaction as indicative of syphilitic infection. 

Bruck Test. A new serum test for syphilis has recently 
been described by C. Bruck.* Following are recent results 
in our laboratory with this test.f 

This new test for the diagnosis of syphilis by C. Bruck 
has aroused much interest. The scientific standing of Bruck 
and the simplicity of the technique led us to overcome 
our prejudice, that has been the offspring of the numerous 
tests that have been offered of late. Bruck states that since 



* Bruck. Miinch. med. Wochen. Jan. 22, 191 7. 
t Smith and Solomon. Boston Medical and Surgical 
Jour. 



480 APPENDIX A 

the discovery of the complement fixation test for syphilis by 
Wassermann, Neisser and himself in 1906, he has been trying 
to find a simple chemical reaction that would take the place 
of the complicated technique of the Wassermann reaction. 
This method, as he has published it, was worked out and is 
being used at the front, in the present war, where complete 
laboratory equipment is not available. 

Commencing our experiments with a great deal of 
scepticism, we were much surprised at the results obtained, 
which are given below. Whatever may be the final status 
of the test in the determination of syphilis, we feel that there 
is a great deal of interest in the fact that this simple chemical 
reaction does pick out certain differences in the composition 
of blood sera and that apparently a large number of syphilitic 
sera differ in their chemical composition percentage from the 
majority of non-syphilitic sera. 

The technique, while exceedingly simple, offers many 
chances for errors and individual variations so that we have 
thought it well to give directions and cautions at some 
length. 

Bruck's* technique is described as follows : "The test is made 
with 0.5 cc. clear serum in a test tube, to which is added 2 cc. 
of distilled water, and the whole shaken. Then, with a 
precision pipette, 0.3 cc. of the ac. nitr. purum of the German 
pharmacopeia is added and the whole thoroughly shaken and 
then set aside at room temperature for ten minutes. Then 
16 cc. of distilled water at room temperature is added, and 
closing the tube with the finger, it is shaken up and down 
three times carefully, not vigorously enough to make it foam. 
This is repeated ten minutes later, and the tube is then set 
aside for half an hour. By this time the precipitate is 
entirely dissolved in the tube with the normal serum, while 
the syphilitic serum shows a distinct, fiocculent turbidity. 
In two or three hours, or better still, in twelve hours, the 
gelatinous and characteristic precipitate is piled up on the 
floor of the test tube." 

* Bruck: Journal of American Medical Association, Vol. 
Iviii, No. 12, March 24, 1917, p. 944. 



APPENDIX A 481 

The acid Is prepared by diluting the Acidum nitricum of 
the U. S. P. (Sp. gr. 1.403) with distilled water until the 
hydrometer shows the specific gravity 1.149, which corre- 
sponds to the nitric acid of the German pharmacopeia, but 
since this requires a special hydrometer, a simpler method is 
to make a 25 per cent solution of the Acidum nitricum, which 
will give about the proper specific gravity. 

The serum Is obtained by allowing 10 cc. of blood to stand 
at room temperature for an hour, and then centrifuging. 
Serum that has stood for some time may be used as well as the 
fresh, and even bloody serum does not seem to confuse the 
results to any great degree. The serum gives the same 
results with or without inactlvation. Post-mortem blood 
gave results as constant as that obtained during life, In the 
few cases that we had in this series. But the reaction may 
be influenced markedly by the size of the test tubes. We 
have found that the 13 X 1.9 cm. is the most favorable size. 

When one first thinks of this test it appears very simple 
and probably somewhat crude as a chemical reaction, but 
there are certain precautions that must be observed, and 
several hundred normal and syphilitic sera should be tried 
before the Investigator can feel that he has a refined routine 
technique. There Is the personal equation which must be 
watched, for here is probably the greatest source of error, 
and readily explains why two different persons get widely 
varying results with the same sera if they have done only a 
few dozen tests. We must take it for granted that the 
reaction is a quantitative one, where some positive reactions 
may differ only slightly from the normal non-syphilitic, and, 
furthermore, any normal serum may be made to give a positive 
reaction, and almost any positive serum be made to give a 
negative by improper manipulation at some point in the test. 
There are as many places for error to creep in as there are 
steps in the process. Bruck has omitted many details in 
his publication, which allow personal variations, and so we 
have tried to develop a routine process that will eliminate 
as many of these as possible. 

We shall here attempt to explain the methods which we 
have found most satisfactory and at the same time indicate 



482 APPENDIX A 

the places where error is likely to occur. The 0.5 cc. of 
serum is added to 2 cc. of distilled water, and shaken thor- 
oughly. Now add slowly exactly 0.3 cc. of acid from a 
precision pipette, care being taken it does not flow down the 
side of the tube. The tube should be shaken gently while the 
acid is being added, for this prevents the formation of a 
flocculent precipitate in normal serum which is difficult to 
dissolve later. After the acid is added shake each tube 
gently to make sure that these flakes do not persist. It is 
difficult to shake each tube in exactly the same manner, as 
must be done if we expect uniform results. 

The first 250 tests of this series were made by allowing the 
tubes to stand for ten minutes as Bruck advocates. Then we 
found that practically all sera gave a positive reaction if 
allowed to stand 15-20 minutes, and so in the other tests of 
the series an attempt was made to make the reaction more 
sensitive by allowing the tubes to stand only 6-7 minutes. 
During this time the tubes should be shaken gently once or 
twice. The manner in which the 16 cc. of water is added also 
influences the reaction. If allowed" to flow freely in upon 
the precipitate, the positive may be forced into solution as 
well as the negative. Both pipette and tube should be 
slanted and the water allowed to flow down the side of the 
tube without disturbing the precipitate. If all has gone well 
up to this point, we may see a marked difference between the 
normal and syphilitic precipitates, in that the normal will 
begin to go into solution at once, thus clouding the water, 
while a positive precipitate will be composed of large flakes 
which show little or no tendency to go into solution or cloud 
the water above. It must be remembered that the most 
flocculent positive precipitate will go into solution if the fluid 
is splashed or shaken too hard while the tube is being inverted. 
If any doubt as to the character of the precipitate now exists, 
it may be allowed to stand ten minutes longer, and again 
inverted as before, or even repeated several times during the 
next hour or two. We see no reason why the tubes should be 
left to stand over night, for during this time a precipitate 
usually settles in the normal tubes. This, however, differs 
from the syphilitic precipitate in that it is still finely granular 



APPENDIX A 483 

and goes back into solution readily when the tubes are 
inverted. 

In view of these possible grounds for error, it is only 
logical to run controls of known positive and known negative 
sera along with each group of unknown bloods, and even then 
certain tubes will seem doubtful, in which event the test 
should be repeated with added precaution to see if a definite 
positive or negative reaction may be obtained. 

In the last tests of this series we seemed to aid the reaction 
by rendering the serum-water solution alkaline by one or two 
drops of 10 per cent potassium hydroxide before the acid was 
added. The positive sera have a larger precipitate, while 
the normal seem to dissolve more readily. 

Table I 
Syphilis: nervous system involved. 

iWassermann and Bruck agree positively 47 

" " negatively 7 

" " at variance 10 

Tabes Dorsalis | Wassermann and Bruck agree positively 3 

P , • 1 i Wassermann and Bruck agree positively 8 

1 " " negatively 3 [ 

Juvenile Paresis | Wassermann and Bruck agree positively I 

Summary Wassermann and Bruck agree positively 59 

" " negatively 10 

" " at variance 10 

Table II 
Syphilis: nervous system not involved. 

jj .... f Wassermann and Bruck agree positively 12 

^ I " " at variance 5 

j^ -.11- , f Wassermann and Bruck agree positively 3 

Congenital Syph.-^ „ <. .• 1 

[ negatively 2 . 

Summary: Wassermann and Bruck agree positively 15 

" " negatively _. 2 

" " at variance 5 

Table III 

Non-syphilitic: Wassermann reaction negative. 

Doubtful or positive Bruck 86 

Bruck test negative 216 

Total for three groups: 

Wassermann and Bruck agree positively 74 

. '* " negatively 230 

- " at variance lOi 



484 APPENDIX A 

The tests here reported were made on blood sera obtained 
from patients admitted to the Psychopathic Hospital and its 
Out-Patient Department. As a routine Wassermann test 
is made on each patient who enters the hospital, it was only- 
necessary to take another tube of blood from each patient, 
and check the results in each instance with the Wasser- 
mann reaction. As it takes several days to get the report 
from the Wassermann laboratory of the State Board of 
Health, there was no chance of being prejudiced by a previous 
knowledge of the Wassermann reaction. The cases for the 
most part were those of mental disease; the majority in good 
general physical health. 

A comparison of the total number with the Wassermann 
reaction shows that there was a general agreement of 304 of 
the 405 cases tested, or a percentage agreement of practically 
75%. In considering the cases of syphilis of the central 
nervous system in a group by themselves, we find that the 
agreement is closer, since 69 of the 79 cases tested, or 87% 
agreed without any question of doubt. It will be noted that 
in several cases of general paresis, the Wassermann reaction, 
which was repeated at intervals, was negative, and in most of 
these cases the Bruck test was negative also. Our few cases 
of congenital and latent syphilis also checked very closely 
with the Wassermann test. In the various groups of mental 
cases in this series, no factor of interference was discovered. 
It is also of interest that in the cases where the blood was 
obtained post mortem, the Bruck test agreed with the Wasser- 
mann result obtained on ante-mortem blood serum. Further 
work on post-mortem sera will be reported. Some of the 
patients not included in the syphilitic groups that have a 
negative Wassermann and no clinical signs of syphilis, give a 
history of previous infection at some time, which might partly 
account for the variations in the two tests. 

CONCLUSIONS 

I. We present results of the Bruck sero-chemical test in 405 
cases. In loi of these cases there were definite clinical 
manifestations of syphilis, in which the Wassermann 
and Bruck tests agreed positively in 74 or 75%. The 



APPENDIX A 485 

two tests agreed negatively in 12 instances, and were 
at variance in 15. 

2. In the group which showed syphilis of the nervous system 

we had 64 cases of clinically certain general paresis, of 
which the Wassermann and Bruck tests agreed in 54 
instances, or practically 85%. In other forms of central 
nervous system involvement the agreement was 100% 
in the 15 cases tested. 

3. In the cases with no apparent involvement of the nervous 

system the agreement was somewhat less, being 76%. 
This may be in keeping with the fact that the Wasser- 
mann test was not so strongly positive in these cases. 

4. The advantages of the test are: (i) the short time 

required to do the test; (2) the limited amount of 
apparatus necessary, and (3) the simplicity of the 
technique. 

5. The disadvantages of the test seem, for the most part, 

to be bound up in the personal variations that are apt 
to occur. 

6. We are here dealing, most probably, with a quantitative 

chemical difference in the protein content of syphilitic 
and non-syphilitic sera, the nature of which is not 
understood by us. It is our hope that this may be 
brought to light in the near future in the field of chem- 
istry. 



APPENDIX B 

COMMON METHODS OF TREATMENT USED 
IN CASES OF NEUROSYPHILIS 

The treatment for neurosyphilis according to the viewpoint 
of the authors is treatment for syphilis. It is necessary in 
order to cure a case of neurosyphilis to cure the syphilis in 
the patient. Accordingly, the methods of treatment best 
adapted for the cure of syphilis are indicated in the treatment 
of neurosyphilis. As experience shows that it is often more 
difficult to cure the neurosyphilitic cases, treatment will have 
to be pushed with greater intensity than in some non-nervous 
system syphilis. In general, then, the methods that have 
been applied by the syphilologist will be used in the treat- 
ment of cases of neurosyphilis. In addition, methods at- 
tempting to bring the drug into local contact with the central 
nervous system have been devised. The methods of treat- 
ment have been in part indicated in Chart 27. 

The method chiefly used in treatment of the cases of this 
book is what we have called intensive systematic intravenous 
treatment. The treatment consists of intravenous injections 
of salvarsan (or a substitute for salvarsan, as arsenobenzol 
and diarsenol) given in a dose of about 0.6 gram and repeated 
twice a week over a period of a number of months. In 
addition, injections of mercury salicylate averaging 0.065 
gram once a week are given and potassium iodid by mouth. 
As indicated, the important point is to keep up treatment for 
a long period of time. This method has produced practically 
no untoward results, certainly no more untoward results 
than are to be expected with salvarsan In smaller quantities 
and it has seemed to us that the therapeutic results have 
been as satisfactory as In any other form of treatment. 

Specialized forms of treatment intended to place the drug 
in contact with the central nervous system may be described 

486 



APPENDIX B 487 

under the headings of spinal intradural treatment and cere- 
bral subdural and intraventricular treatment. 

Three main therapeutic agents have been largely used. 
These are (i) salvarsanized serum according to the method 
of Swift-Ellis (in vivo). The serum according to this method 
is prepared as follows: An intravenous injection of salvarsan 
is given to a patient and blood withdrawn at the end of one- 
half hour. This is allowed to clot. The serum is removed 
and after inactivation at 56° C. for one-half hour it is ready 
for use. The average dose is 15 to 30 cc. of serum. As a 
matter of fact, it is not necessary to use the blood serum from 
the same patient to whom the intraspinous injection is to be 
given. (2) The salvarsanized serum according to the method 
of Ogilvie (in vitro). Blood serum is prepared from any 
patient and to it is added salvarsan in such a strength that 
the amount to be injected, 10 to 30 cc. of serum, will contain 
o.oooi to o.ooi gm. (3) Mercurialized serum according to 
the method of Byrnes. Mercury bichloride is added to blood 
serum in such proportion that the amount of serum to be 
injected will contain from 0.00065 gram to 0.0026 gram. 

The method of intraspinous injection is to perform lumbar 
puncture, withdraw an amount of fluid approximately 
equivalent to the amount to be injected; then allow the serum 
to be Injected to run In by gravity. 

For the cerebral, subdural and intraventricular injections, 
the same sera may be used as for the Intraspinous. Five or six 
times as much salvarsan may be given, but a smaller amount of 
serum may be advisable, that is, 10 to 15 cc. To perform Injec- 
tions a trephine opening Is made In the calvarlum about the size 
of a dime. The location of choice for the opening is slightly 
back of the longitudinal prominence just to the right of the 
median line, to avoid the frontal sinus. For subdural injec- 
tions a curved needle is thrust between the dura and the 
brain and the serum allowed to flow In slowly by gravity. For 
the intraventricular injections a blunted spinal puncture 
needle is thrust through the brain substance into the 3rd ven- 
tricle. When the 3rd ventricle is reached the clear cerebral 
fluid will flow out; then after withdrawing a sufficient amount, 
the serum may be introduced by gravity. The trephining may 



488 APPENDIX B 

be done under local anesthesia but as a rule it is better to 
induce general anesthesia. The subsequent injections can 
be made without recourse to any anesthesia whatsoever, as 
they are practically painless. 

All procedures both in the injections and in the preparation 
of sera are naturally to be performed under aseptic conditions. 



INDEX 



Abscess, tonsillar, associated with neu- 
rosyphilis, 250. 
Addison's disease in juvenile paretic, 

279. 
Agraphia, loi. 
Albumin test, 474. 
AUbutt, Clifford, 257. 
Alcoholism, chronic, 227. 
Alcoholic dementia, 237. 

epilepsy, 229. 

hallucinosis, 225. 

pseudoparesis, 222, 223, 451. 
Allergic, 129, 204. 
Alzheimer, 428. 

method, 472. 
Amboceptor, 477. 
Amnesia, 195. 
Anaphylaxis, 129. 
Anatomical formulae, 25. 
Antigens, 476. 
Aortic aneurysm, 35, 439, 

sclerosis, 41, 46, 135. 
Aphasia, 31, 43, loi, 262, 445. 
Apoplexy, 197. 

Argyll- Robertson pupil, 209, 212, 217, 
291, 450. 

as isolated symptom, 217. 

in alcoholism, 214, 229. 
Arndt, Junius and, 249. 
Arsenobenzol, 375, 377, 389, 486. 
Arteriosclerosis, cerebral, loi. 

not a contraindication to intensive 
salvarsan therapy, 359. 

radial, 68. 
Ascending lesion, 23. 
Asymmetrical lesions, 19. 
Ataxia, 31, 223. 

Atheromatous degeneration, 35. 
Atrophy, cerebellar, 39. 

cerebral, 47, 134, 205. 

parenchymal, 41. 

pontine, 39. 



Atypical case congenital neurosyphilis 

270, 
Ayer, J. B., 472. 

Ballet, 72. 

Barrett, A. M., 54, 175, 187, 212, 218, 

219. 
Bechterew, 219. 
Binet and Simon, 304. 
Binet scale, 277. 
Birnbaum, 403. 

Blood pressure, high, 70, 262, 124. 
Bly, 252. 

Bonhoeflfer, 404, 415, 417. 
Bordet, 427. 
Bratz, 278. 
Bruck test, 479. 
Bruck, C, 479. 
Bumke, 214, 

Canavan, 256. 
and Southard, 70. 

Cell count, 471. 

Cerebral syphilis, see diffuse neuro- 
syphilis. 

Cerebrospinal syphilis, see diffuse 
neurosyphilis. 

Cervical hypertrophic meningitis of 
Charcot, 56, 441. 

Chancre, extragenital, 75, 342. 

Character change, neurosyphilis, 314. 

Charcot, 60, 186. 

Choroiditis, 242. 

Christian, 407. 

Cimbal, 403. 

Civilization and syphilis, 76. 

CHnical evidences of syphilis, 131. 

Clouston, 158. 

Collins, Joseph, 145. 

Compensation in neurosyphilis, 309, 
402, 456. 

Complement, 477. 



489 



490 



INDEX 



Conduct disorder, 38. 
Congenital syphilis, absence of stig- 
mata, 318. 

as cause of feeblemindedness, 159,447. 

involvement of nervous system in, 

274. 
Congenital neurosyphilis, 270, 395. 

resembling feeblemindedness, 272. 
Conjugal neurosyphilis, 263. 
Convulsions, 43, loi, 248, 362. 

cause of in paretic neurosyphilis, 232. 

in psychopathic subject with 
syphilis, 417. 
Corneal opacity, syphilitic, 234. 
Cotard, 73. 
Cotton, H. A., 472. 
Craig, C. B., 152, 196. 
Cramer, 125. 
Cranial neurosyphilis, 140. 

tenderness, 139. 
Crises, gastric, 367, 
Cysts, ependymal, 59. 

of softening, 27, 36, 54. 
Cytorrhyctes luis, 381. 

Dana, Charles L., 65, 77, 78. 
Dazed states, 264. 
Deafness, 63. 
Decompression, 138. 
Defective delinquent — diffuse neuro- 
syphilis, 300, 455. 
Dejerine-Tinel, 61. 
Delinquency and juvenile neurosyphilis, 

298. 
Delirium tremens, 332. 
Dementia, 137. 
Dementia paralytica, see paretic 

neurosyphilis. 
Dementia praecox, 74, 185, 247. 
Depression, 95, 126. 
Depressive drugs, 189. 
Diabetes, and neurosyphilis, 240. 

insipidus, 190. 
Diabetic pseudoparesis, 238. 
Diarsenol, 377, 389, 391, 486. 
Differential diagnosis, alcoholism and 
neurosyphilis, 227, 231, 234, 236. 
brain tumor, diabetic pseudoparesis 

and neurosyphilis, 238. 
diffuse and paretic neurosyphilis, 
165, 193, 247. 



Differential diagnosis, manic-depressive 
psychosis and neurosyphilis, 69. 
multiple sclerosis and neurosyphilis, 

253, 255. 
neurasthenia and neurosyphilis, 65, 

183. 
senile arteriosclerotic psychosis and 
neurosyphilis, 262. 
Diffuse neurosyphilis, cerebrospinal 
syphilis, cerebral syphilis, spinal 
syphilis, 17, 80, 85, 97, 103, 122, 
140, 183, 193, 300, 331, 342, 359, 
433, 439, 443- 
premonitory symptoms, 342. 
prognosis, 80, 103, 124, 433, 443. 
spinal fluid findings in, 348. 
symptoms, 99. 

treatment, 98, 103, 184, 302, 390. 
treatment, results, 343. 
Diplopia, 50, 184, 253, 356. 

causes, 140. 
Donath, 401, 403. 
Drastich, 407. 
Duco and Blum, 403. 
Dupre, 407. 
Dysdiadochokinesis, 231. 

Ehrlich, 184, 428, 429. 
Encephalitis, 27, 248. 

disseminated, 218. 
Endarteritis, 220. 
Ependymal cysts, 59. 
Ependymltis, 40, 47, 49, 134. 
Epilepsy, 192. 

alcoholic, 229. 

brought out by syphilis, 415. 

Jacksonlan, 103. 

parasyphilitic, 194. 

relation to j u venile neurosyphilis, 277. 

syphilitic, 103, 194. 

syphilogenic, 415. 
Epileptic neurosis, 195. 
Erb's syphilitic spastic paraplegia, 147. 

treatment of, 148. 
Euphoria, 73. 
Excited states, 95. 
Exner, M. J., 416. 

Exophthalmic goitre, syphilitic (?), 205. 
Extraocular palsy, 140, 441. 
Eye changes In neurosyphilis, 257. 
Eye muscles, paresis of, 17, 50. 



INDEX 



491 



Facial paralysis, 53. 

Families of neurosyphilitics, 275, 316, 

318,320,373,431,457. 
Family of neurosyphilitic, normal look- 
ing, but syphilitic, 318. 
Familial syphilis, 299, 306. 
Farrar, C. B., 411. 
Fearnsides, Head and, 21, 140, 150, 

193. 217, 374. 378. 
Feeblemindedness, 395. 

and congenital syphilis, 159, 
Fernald, W. E., 159, 273, 396. 
Fildes, Mcintosh and, 129, 329. 
Focal changes, 221. 

meningitis, 50. 

softenings, pontine, 54. 
Fournier, 142, 222, 186, 194, 381. 
Franz, 357. 
Froissart, 413. 
Fugue, hysterical, 264. 

Garnier, 407. 

General paresis, see paretic neuro- 
syphilis. 
Glands, 270. 

Gliosis, 39, 47, 49, 136, 180. 
Globulin, 229. 

tests, 473. 
Glycosuria, 238, 241. 
Goddard, 397. 
Gold sol reaction, 247, 474. 

in brain tumor, 100. 

paretic, 85, 98. 

paretic, other tests negative, 383, 385. 

in purulent meningitis, loo. 

syphilitic, 85, 98, 345. 
Graham, Thomas, 429. 
Grandiosity, 72, 295, 455. 
Graves, W. W., 157. 
Grille, 407. 
Gross, 257. 

Gumma, see gummatous neurosyphilis. 
Gumma of tonsil, 250. 
Gummatous neurosyphilis, 53, 56, 137, 
138, 140, 221, 362, 438. 

Hallucinations, 53. 

in paretic neurosyphilis, 249. 
Hauptmann, 348. 

Head and Fearnsides, 21, 140, 150, 193, 
210, 217, 374, 387. 



Headache, 53, 63, 122, 247, 352. 

causes of, 209. 
Hecht, 399. 

Hemianopsia in neurosyphilis, 242. 
Hemiplegia, 31, 45, 80, 122, 262, 360. 

causes of, 389. 
Hemitremor, 197. 
Heredity, neuropathic, 84. 
Herxheimer reaction, 152. 
Heubner, 427, 428. 
Hinton, W. A., 471. 
Huntington's chorea, 258. 
Hutchinsonlan teeth, 45. 
Hydrocephalus, 134, 306. 
Hyperreflexia, explanation of, 233. 
Hypochondriacal ideas, 133. 
Hysteria, 815, 301. 
Hysterical symptoms, 18. 

Incontinence, vesical in tabetic neuro- 
syphilis, 144. 

rectal, 56. 
Incubation period of neurosyphilis, 152, 
Infectiousness of neurosyphilis, 95. 
Insight, 95. 
Insomnia, 63. 

Intracranial pressure, 139, 362. 
Intraspinal lesions, 95. 
Intraspinous therapy, 122, 366, 486. 

unpleasant results of, 366. 
Intraventricular Injections, 389, 487. 
Involution melancholia, 187. 
Iodine, untoward results, of, 363. 
Iritis, 17. 

Jarisch-Herxheimer reaction, 72. 
Joffroy, 214. 

and MIgnot, 64. 
Junius and Arndt, 249, 
Juvenile neurosyphilis, 438, 447. 

relation to epilepsy, 277. 
Juvenile paresis, see juvenile paretic 

neurosyphilis. 
Juvenile paretic neurosyphilis, juvenile 
paresis, 45, 154, 157, 272, 275, 298, 
306, 440. 

age of onset, 158. 

and Addison's disease, 279. 

and delinquency, 298. 

prognosis, 156, 158, 162, 273, 275. 

treatment, 154, 161, 278, 299. 



492 



INDEX 



Juvenile paretic neurosyphilis, with 
initial trauma, 306. 
congenital amputation of toes in, 158. 
Juvenile tabetic neurosyphilis, 161, 

447. 

Kaplan, 255, 471. 

Keraval, 257. 

Key, 427. 

Knee-jerks, absence of, 223. 

lively, 75. 

return of, 24. 
Koefod, Solomon and, 243. 
Kolmer, 471. 
Kraepelin, 65, 66, 69, 88, 91, 95, 187, 

225, 249. 
Krafft-Ebing, 84. 

Laignel-Lavastine, 413. 

Lange, C, 428, 429, 474. 

Lancinating pains, 92, 141. 

Lepine, 408, 413. 

Leptomeningitis, 47, 54, 135, 

Lewandowski, 210. 

Liability of paretic, 295. 

Lissauer's paralysis, 38. 

Locomotor ataxia, see tabetic neuro- 
syphilis. 

Long, 418. 

Lucke, Baldwin, 93, 144, 

Lues maligna, 250, 452. 

Lumbar puncture, untoward eflfects, 
352. 
treatment of, 354. 

Liith, 278. 

Lymphocytosis, 23, 30, 40, 49. 

McDonagh, 381. 

Mcintosh, Fildes and, 129, 329, 

Malaria, cerebral, simulation of paretic 

neurosyphilis, 245. 
Mallory and Wright, 472. 
Manic-depressive psychosis, 68, 71, 77, 

187, 202, 291, 384, 442. 
Marie, Chatelin and Patrikios, 412. 
Marie, 408, 414. 
Martin, E. G., 313. 
Massary, de, 414. 
Mattauschek and Pilcz, 347. 
Medicolegal and Social, 454. 

period of paretic neurosyphilis, 414. 



Meilhon, 407. 
Memory, failing, 63. 
Meningitis hypertrophica cervicalis of 
Charcot, 56. 

sympathica, 19. 

syphilitic, 103. 
Mercurialization, 98. 
Mercury, 58, 83, 85, 98, 148, 193, 235, 
376, 377, 389, 391, 395, 486. 

untoward results of, 363. 
Metasyphilis, 89. 
Metchnikoff and Roux, 427, 428. 
Microgyria, occipital, 47. 
Mignot, JofTroy and, 64, 66. 
Migraine, 19. 
Mitchell, H. W., 218. 
Moebius, 429. 

Mott, F. W., 158, 257, 308, 396, 437. 
Multiple sclerosis, 253, 256, 

relation of syphilis to, 254. 

spinal fluid findings in, 254. 
Muscular atrophy, 149, 446. 

syphilitic relation to amyotrophic 
lateral sclerosis, 150. 
Muscular weakness, 279. 
Myerson, A., 196. 

Nageotti, 428. 

Nausea, 63. 

Neisser, 399. 

Nerve trunk tenderness, 148, 234. 

Nervousness, 63. 

Nervous indigestion, 63. 

Neurasthenia, 63, 183. 

Neuritis, cranial, 51. 

optic, 365. 

root, 235. 

syphilitic, 235. 
Neurorecidive, 152, 153, 184, 196, 235. 
Neuroses, relation of syphilis to, 186. 
Neurosyphilis, 187, 238, 240, 242. 

aggravated on military service, 404. 

atypical, 258, 346. 

atypical case resembling hysterical 
fugue, 264. 

dates, 428. 

forms of, 20, 21, 28, 29, 95. 

galloping, 328. 

history of, 427. 

incubation period, 152. 

infectiousness of, 95. 



INDEX 



493 



Neurosyphilis, laboratory findings in, 
82. 

latent, 142, 203. 

lesions, 303, 

lighted up by stress of military serv- 
ice, 412. 

and marriage, 319. 

prevention, 320. 

onset, 64. 

in primary stage, 186. 

in secondary stage, 185, 283, 390. 

in secondary stage, prognosis, 390. 

in secondary stage, treatment, 153. 

spinal, 23. 

and the war, 399, 466. 
Nissl-Alzheimer method, 427. 
Noguchi, 381. 

and Moore, 428, 429. 
Nonne, 82, 125, 152, 186, 195, 196, 214, 
216, 235, 254, 265. 

-Apelt test, 473. 
Numbness, 56. 
Nystagmus, 45, 253, 256, 279. 

Obersteiner, 249. 
Occupation neurosis, 312. 
Ogilvie method, 487. 
Operation for gumma, 139. 
Optic atrophy, 256. 

in juvenile paretic neurosyphilis, 154. 
Optic thalamus, syphilitic lesion of, 

205. 
Osteitis, syphilitic, 311. 
Ozena, 350. 

Pains, 31. 

Pandy test, 474. 

Paralysis, 123. 

recovery from, 342. 
of respiration, 248. 

Paranoia, syphilitic, 225. 

Paraphasia, 19, 43. 

Paraplegia, 26, 30. 

Parasyphills, 89, 

Paresis sine paresi, 126, 186, 204, 303, 
445. 

Paresis, see paretic neurosyphilis. 

Paretic neurosyphilis, dementia para- 
lytica, general paresis, softening of 
the brain, 37, 63, 68, 74, 78, 80, 85, 
97, 131, 188, 192, 197, 199, 202, 



227, 241, 262, 289, 295, 309, 314, 
323, 338, 372, 375, 377, 382, 384, 
386, 388, 392, 435, 440, 442. 

adjuvant causes of, 414. 

causing social complications, 289. 

causes of death in, 197. 

course, 85. 

duration, 88. 

forms, 95. 

Improvement, 377. 

incidence among officers, 407. 

Incidence among soldiers, 402. 

lesions of, 131. 

"lighted up" by domestic stress in 
civil life, 420. 

"lighted up" by "gassing," 414. 

mortality from, 89. 

nomenclature, 88. 

onset, 192. 

pathology of, 436. 

prognosis, 435, 444. 

symptoms, 90, 131. 

symptoms, mental, 87. 

symptoms, physical, 86. 

versus diffuse neurosyphilis, 165. 

versus vascular neurosyphilis, 169, 
172. 

with very marked meningitis, 332. 

with very marked brain atrophy, 335. 

without mental symptoms, 315. 

traumatic exacerbation, 310. 

traumatic form, 308, 413. 

traumatic, shell shock, 401. 

treatment of, 85, 370, 372, 377, 382, 
384, 386, 388, 392. 

treatment, results of, 351. 
Pensions for disabilities resulting from 

venereal disease, 409. 
Pensions for neurosyphilis, 411. 
Peripheral neurosyphilis, 19. 
Perivascular Infiltration, 41. 
Pernicious anemia with spinal symp- 
toms, 267. 
Petit mal attacks, 195. 
Pforringer, 61. 
Phobia, 67. 

PIlcz, Mattauschek and, 347. 
PItres and Marchand, 421, 424. 
Plant, 249, 348, 428. 
Plaut, Rehm and Schottmiiller, 471. 
Plasmocytosis, 40, 49, 55, 



494 



INDEX 



Pleocytosis, 23, 220, 247, 344, 

effect of antisyphilitic treatment on, 
244, 376. 

in remissions, 243. 

significance of, 243. 

spinal fluid otherwise negative, 270. 
Polydipsia, 190. 
Polyuria, 190. 
Pontine hemorrhage, 219. 

softening, 54. 
Posey and Spiller, 257. 
Potassium iodid, 58, 85, 98, 193, 222, 

376, 377, 389, 486. 
Preparesis, 65, 77, 78. 
Prince, Morton, 195. 
Psammoma, 213. 
Pseudoneurasthenia, 66. 
Pseudoparesis, 449. 

alcoholic, 222, 229, 451. 

diabetic, 238. 

senile, 263. 

shell shock, 421. 

syphilitic, 223, 371. 
Pseudoparetic neurosyphilis, 222. 
Pseudotabes, shell-shock, 424. 
Psychogenic neurosyphilis, 189. 
Psychographic disturbance, 228. 
Psychopathic personality, 302. 
Ptosis, 350. 
Pupillary reaction, changes in, 261. 

signs, 69. 
Pupils, Argyll-Robertson, see Argyll- 
Robertson pupils. 

irregular, 79, 201. 

normally reacting in paretic neuro- 
syphilis, 199. 

sluggish reaction to light, 188. 

stiff as isolated symptom, 265. 
Purkinje cells, binucleate, 48. 
Putnam, James J., 19, 56. 
Pyramidal tract lesion, bilateral, 326. 

sclerosis, 44. 

Quadriplegia in juvenile paretic neuro- 
syphilis, 275. 
Quincke, 427, 428. 

Randsklerose, 24. 

Ravaut, 428. 

Ravaut, Sicard, Nageotti, Widal, 428. 

Rayneau, 407, 413, 414. 



Recovery, 77. 

Recurrences, 70. 

Redlich, 403. 

Regis, 73. 

Remissions, 122, 435, 445. 

Retardation, 187. 

Retention of urine, 56. 

Retinitis, hemorrhages, 365. 

Richards, R. L., 402, 404, 406, 409. 

Robertson, A. R., 59. 

Rod cells, 226, 297, 

Romberg sign, 141, 216, 279. 

Root sciatica, syphilitic, 418. 

Rosenau, 471. 

Ross-Jones test, 473. 

" Rum fit," 229. 

Ryder, Charles T., 42. 

Saddle-shaped nose, 210. 
Salivation, 98. 
Salmon, Thomas W., 89. 
Salvarsan, 75, 83, 85, 193, 222, 377, 
389, 486. 

provocative, 78, 79. 

untoward results of, 363. 
Salvarsanized serum, 75. 
Schaudinn, 427, 429. 
Sciatic pain in neurosyphilis, 149. 
Seizures, 31, 64, 83, 103, 444. 

causes of in paretic neurosyphilis, 
194. 

Jacksonian, 392. 

minor, 392. 
Senile arteriosclerotic psychosis, 262. 
Sensitized cells, 478. 
Serieux and Ducaste, 96. 
Shaikewicz, 404. 
Shanahan, 278. 
Sheep's corpuscles, 477. 
Shock, 42, 81. 
Sicard, 428. 
Six tests, 80, 85. 

In tabetic neurosyphilis, 141. 
Smith and Solomon, 479. 
Social cases, 454. 

service, 232. 
Solomon, 142, 255. 

and Koefod, 243. 

Smith and, 479. 

Southard and, 202, 303. 
Somnolence, 45. 



INDEX 



495 



Southard, E. E., 48, 134, 212. 

and Canavan, 70. 

and Solomon, 202, 303. 

and Taft, 397. 
Spasms, clonic, 326. 
Spastic hemiplegia in paretic neuro- 
syphilis, 323. 
Spastic paraplegia, Erb's, 147, 306. 
Spasticity, 18, 256. 
Speech defect, 69, 133. 
Spiller, 150. 

Posey and, 257, 
Spinal fluid findings in secondary stage 
of syphilis, 151, 185, 283. 

in juvenile paretic neurosyphilis, 275. 

negative in diffuse neurosyphilis, 140. 

negative in gummatous neuro- 
syphilis, 138. 

negative in neurosyphilis, 2 1 6. 

negative in tabetic neurosyphilis, 
269. 

in tabetic neurosyphilis, 141. 
Spinal fluid, withdrawal for therapeutic 

purposes, 377, 379. 
Spinal syphilis, see diffuse neuro- 
syphilis. 
Spirochetes, "drug fastness," 381, 394. 

strains, 76, 263, 276, 381, 394. 
Steida, 405. 

Sterility in tabetic neurosyphilis, 1 44. 
Stier, 407. 

Stokes, Wile and, 186. 
Suicide, 92, 126, 240, 296, 301. 
Summary, 427. 

Syphilis aggravated by service, 406, 
411. 

on service, 409. 
Syphilis as cause of diabetes, 241. 

as cause of feeblemindedness, 396. 

hereditaria tarda, 160, 318. 

history of, 427. 

lesions in, 329. 

of lung, 211. 

from Mongolian, 76. 

primary, 65. 

secondary, 65. 

tertiary, lesions in, 329. 
Syphilitic feeblemindedness, pathology 
of, 160. 

neuritis, 312. 

psychosis, 91. 



Syphilophobia, 67, 361. 
Syphilotoxins, 72. 
Swift, 129, 212. 
Swift and Ellis, 428, 429. 
method, 428, 487. 

Tabes dorsalis, see tabetic neuro- 
syphilis. 
Tabetic neurosyphilis, tabes dorsalis, 
locomotor ataxia, 30, 31, 141, 146, 
366, 367, 434, 446. 
associated with cerebral symptoms, 

177. 
atypical, 143. 
cervical, 146. 
course, 141. 
with negative spinal fluid findings, 

269. 
prognosis, 94. 
shell shock, 403, 

"shell shocked" into paretic neuro- 
syphilis, 401. 
symptoms, 93. 

symptoms in order of frequency, 145. 
treatment, 145, 366, 367. 
plus vascular neurosyphilis, 175. 
with vascular insult, 30, 439. 
versus pernicious anemia, 267. 
Taboparesis, see Taboparetic neuro- 
syphilis. 
Taboparetic neurosyphilis, taboparesis, 
92, 135, 195, 284, 443. 
course, 92. 
nomenclature, 94. 
prognosis, 92, 443. 
and typhoid meningitis, 284. 
Taft, A. E., Southard, E. E., and, 
Talon, 407. 
Taylor, E. W., 50. 
Temperature, paretic, 376. 
Tests, changes under treatment, 102. 
changed to negative in paretic neuro- 
syphilis without clinical improve- 
ment, 385. 
changed to less strongly positive in 
paretic neurosyphilis without clini- 
cal improvement, 386. 
Therapeutic conception, 324. 
Thibierge, 399. 
Thierry, 158. 
Throbbing in head, 63. , 



496 



INDEX 



Thrombosis, cerebral, 36, 42, 342, 357, 

360, 124. 
Thymus, persistent, 282. 
Tibial exostoses, 100. 
Tigges' formula, 248. 
Todd, J. L., 406, 409. 
Transient deafness, 18. 
blindness, 18. 
paralysis, 124. 

paralysis, condition in which occurs, 
123. 
Trauma and juvenile neurosyphilis, 
278, 306. 
neurosyphilis, 456. 
paretic neurosyphilis, 199, 308, 310. 
syphilitic osteitis, 311. 
Treatment of neurosyphilis, 67, 75, 83, 
124, 148, 184, 222, 235, 299, 328, 
332, 335. 342, 346, 350, 351, 355, 
359, 365, 366, 370, 372, 375, 382, 
384, 390, 392, 395, 419, 439, 457- 
case in which theoretically of no 

avail, 323. 
methods, 356, 486. 
Treatment of syphilis, effect on de- 
velopment of neurosyphilis, 142, 

347. 
Tremor, 197. 

intention, 256. 
Tubercle, 80. 

Tuberous sclerosis of Bourneville, 47. 
Tumor, cerebral, 53, 191, 238, 253. 

pineal, 213. 

Unconsciousness, 53. 
causes of, 389. 

Vascular changes, 220. 
Vascular neurosyphilis, 31, 42, 72, 296, 
359, 433, 440- 



Vascular neurosyphilis, plus tabetic 
neurosyphilis, 175. 

prognosis, 433. 

versus paretic neurosyphilis, 169, 172. 
Veeder, B. S., 274. 
Vertigo, 122. 
Viet, 278. 
Virchow, 427, 428. 
Vomiting, 53, 63. 

Warthin, 241. 
Wassermann reaction, 191. 

and alcoholism, 230. 

in congenital syphilis, 160, 271. 

meaning of "doubtful," 360. 

negative in diffuse neurosyphilis, 184. 

negative in juvenile paretic neuro- 
syphilis, 298. 

negative in spinal fluid in spinal 
syphilis, 148. 

negative in spinal fluid in neuro- 
syphilis, loi. 

negative in neurosyphilis, 252. 

negative in paretic neurosyphilis, 77. 

technique, 476. 

titrations in spinal fluid, 348. 
Wassermann, Neisser and Bruck, 428. 
Weiler, 214. 
Weygandt, 403, 404. 
Widal, Sicard, Ravaut, 428. 
Wiles and Stokes, 186. 
Word deafness, 35, 43. 

X-ray diagnosis of bone conditions, 136. 

Yerkes-Bridges, 304. 

Ziehen, 409. 
Zsigmondi, 429, 474. 



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